Rheumatology
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| What is rheumatoid arthritis (RA)? | It is the most common inflammatory rheumatic disease (causes classic rheumatism) The prototype of auto-immune disease, typically affects small and medium sized joints symmetrically It is a chronic destructive disorder, treated to avoid destruction and inflammation. |
| How is the pathogenesis of RA? | It causes cartilage breakdown, bony erosion and loss of function ultimately in some joints. Primary lesion is synovitis, the leading to the formation of inflammatory pannus. Causes systemic manifestations and reduces life expectancy |
| How is the epidemiology of RA? | 1-3 % of europians, regional variation (acc to genetics and environment) Women are three times more likely to get it. Variable course of action (execerbation and remission vary from one person to another) |
| What are the auto-antibodies seen in RA? | Rheumatoid Factor (RF): targets Fc of IgG, not very specific but sensitive (60-70%) Anti-Citrullinated Peptide Ab (ACPA/ Anti CCP): Attacks citrullinated aa at peptides (arginine), occurs in lungs due to smoking and causes RA, These Ab are highly specific for RA only and is as sensitive as RF. Can be detected pre clinical RA, confirms RA |
| How is the pathobiology of RA? | Genetic predisposition (HLA) with environmental factors (smoking Increase ACPA, RF, TB) to give pre RA seen by elevated ACPA, then a trigger occurs (trauma, infection...) that leads to an inflammatory response triggering RA to start |
| What are the risk factors that give RA? | Genetics (60% heritability, ACPA + or HLADR4/DR1, it is a polygenic disease) Smoking (most common factor, more difficult, erosive and severe RA, induces protein citrullation) Periodontal disease (oral flora imp to take care of oral hygiene) Other factors (coffee, mineral oils, silica dust, alcohol, pregnancy) |
| How is the physiopathology of RA? | APCs present ACPA, then go to T cells and stimulates formation of ACPA Ab, activating inflammatory cytokines Another mechanism is by Ag-Ab complexes accumulating in the joint increasing synovitis In addition, increased cytokines start inflammation, angiogenesis and osteoclastic stimulation thus increasing bone resorption systematically |
| What are the anti-inflammatory cytokines? | TGF-beta, IL10 |
| What are the clinical aspects of RA? | Articular Manifestations: swelling of polyarticular small joints (PIP, MCP, wrist, MTP, TMJ but never DIP) To test swelling, touch all joints squeeze them and touch lateral edges see consistency Extra Articular manifestations (fever, chills, fatigue, anorexia, weight loss, Sicca syndrome (dry eyes and mouth), carpal tunnel syndrome (due to compression on median nerve by ACPA), pericarditis and pleural effusion) Most important is lung and eye involvement |
| What are lab manifestations of RA? | CBC (leukocytosis with high CRP/Erythrocyte sedimentation rate (ESR) and thrombocytosis) High RF and ACPA (the higher the more severe) reduced hemoglobin is common (called anemia of chronic disease) |
| What are image findings in RA? | X ray (gold std for diagnosing damage in RA, 1st sign is soft tissue swelling, perarticular osteopenia, erosions joint space narrowing (which is diagnostic even if negative labs). Erosions occur rapidly 50% first year and 70% 5 years, important to know that first erosion is at MTP5, MTP before MCP. But X ray is limited since it needs 2 years to be visible) US (of hands and feet, earlier detection of erosion, shows joint, inflammation, tendon, angiogenesis, synovium, early detection subclinical synovitis, important to know common presentation is extensor carpi ulnaris tenosynovitis which is pathogneumonic) Others (MRI for early dx and staging not routinely used) |
| How are classifications of RA? and differential diagnosis? | Use ACR/EULAR classifications, not for diagnosis usually, score>6. differential (Osteoarthritis, peripheral SPA, viral arthritis (parvo), Lyme, acute sarcoid (Lofgren), Connective tissue disease (SLE, myositis, Sjogren), crystal, polymyalgia) |
| What are prognostic factors of RA? | Prediction of future structural damage (ACPA, erosive, female gender, acute phase reactants, RF , TNF and IL6, genetic markers (HLADR4), HAQ score, MRI) Prediction of increased mortalitiy (age, education, physical status, RF, comorbidities, infection, extra-articular manifestations, increased HAQ, poor health, glucocorticoud use) |
| How are the clinical aspects of chronic RA? | When we have long lasting inflammation (chronic RA), we will get deformities (Boutonniere, Z shape, A swan neck, ulnar drift, volar subluxation) mainly in MCP, PIP and DIP. We get involvement of C1-C2 (antlantoaxial subluxation) due to synovial mmb between C1 and C2, this is rare and severe life threat, we get persistent synovitis produce erosion at dens and transverse ligament lead to instability, which is risk of rupture and anterior displacement of the dens compressing the spine which may lead to death. As for lower limbs we should check for deformities in MTPs, malate, hallus valgus, subluxation of foot arch, pennus nodules on extensors (rheumatic nodules in seropositive disease). Other joints shoulder elbows TMJ cricoarythenoid, see bursitis or flexor tenosynovitis) |
| What are extra articular manifestations of RA? | Hematology (Anemia, thrombocytosis, Felty's syndrome [splenomegaly w/leukopenia and thrombocytopenia]) Ocular (keratoconjuctivitis sicca, scleritis/episcleritis) Hepatic abnormality, Pulmonary (Pleural effusion, intersitial fibrosis, nodules, alveolitis, dilatation, pneumoconiosis, pulmonary HTN, bronchiolitis) Skin (SC nodules, pyoderma gangrenosum, vasculitis rash, leg ulcer, amyloidosis) Vasculitis (nail fold, systemic) Cardiac (pericarditis, pericardial effusion, valvular heart disease, conduction defects) Neurological (nerve entrapment, cervical myelopathy, neuropathy peripheral or mononeuritis) Infection, Osteoporosis, Muscle wasting, Cancer, Secondary Sjogren, Atherosclerosis, Mortality. Functional disability and consequences (pain and joint destruction, lead to loss of mobility, psychosocial functioning, mental distress, depression and fatigue |
| What is reactive arthritis? | Aseptic arthritis occurs 1 month of a primary infection, acute, assymetric oligoarthritis Extra-articular (ocular inflammation, enthesitis/mucocutaneous lesions [Balanitis circinata ring shaped skin on glans of penis/ keratoderma blennorhagia], Urethritis, Rarely Carditis) Duration 4-5 months of arthritis, recurrent attacks, chronic/recurrent peripheral arthritis, sacroilitis, or spondylitis. |
| What is spondyloarthritits? | Heterogenous group of chronic inflammatory arthropathies affecting mainly the spine with some peripheral symptoms in enthesis and certian extra-articular sites. Enthesis is distiguishing feature of SPA, associated with HLAB27. Sero - for RF and ANA. Important priniciple if pt comes with pain waking him/ at morining we have inflammatory pain, if we have a swollen finger either arthritis, dactylitis or spondyloarthritis. Here we have spine involvement |
| What is reactive arthritis? | Aseptic arthritis occurs 1 month of a primary infection, acute, assymetric oligoarthritis Extra-articular (ocular inflammation, enthesitis/mucocutaneous lesions [Balanitis circinata ring shaped skin on glans of penis/ keratoderma blennorhagia], Urethritis, Rarely Carditis) Duration 4-5 months of arthritis, recurrent attacks, chronic/recurrent peripheral arthritis, sacroilitis, or spondylitis. |
| What is reactive arthritis? | Aseptic arthritis occurs 1 month of a primary infection, acute, assymetric oligoarthritis Extra-articular (ocular inflammation, enthesitis/mucocutaneous lesions [Balanitis circinata ring shaped skin on glans of penis/ keratoderma blennorhagia], Urethritis, Rarely Carditis) Duration 4-5 months of arthritis, recurrent attacks, chronic/recurrent peripheral arthritis, sacroilitis, or spondylitis. |
| What are the types of spondyloarthropathies? | Ankylosing spondylitis, Juvenile chronic arthritis, Psoriatic arthritis and reactive arthritis (these are the most common) We also have arthritis associated with IBD, and undifferentiated SpA |
| What is reactive arthritis? | Aseptic arthritis occurs 1 month of a primary infection, acute, assymetric oligoarthritis Extra-articular (ocular inflammation, enthesitis/mucocutaneous lesions [Balanitis circinata ring shaped skin on glans of penis/ keratoderma blennorhagia], Urethritis, Rarely Carditis) Duration 4-5 months of arthritis, recurrent attacks, chronic/recurrent peripheral arthritis, sacroilitis, or spondylitis. |
| What are clinical aspects of SpA? | According to subgroup (axial or peripheral, Psoriatic or reactive (infection)) Manifestations (Rheumato: axial involvement, peripheral arthritis, enthesopathy. Extra-articular: Acute anterior uveitis (eye inflammation) and cardiac involvement We have a genetic background: HLAB27 and family hx, and specific manifestations: psoriasis and IBD... |
| How is epidemiology of SpA? | Most common one is ankylosing spondylitis (AS) 60% have + HLAB27 (except undifferentiated only 25%) however chance of pt w/ + HLAB27 to get SpA is 1-5% could get to 20% if a relative has it. More common in males (except psoriatic where equal proportions) Mean age 26 years from late adolescence to 40 years, we usually have delay in dx in HLAB27 - pt. |
| How is pathogenesis of SpA? | Genetics (very tricky, first degree relatives increase risk, HLAB27 role in MHC I, microbes which can cross react with B27 Ab to bacteria this is known as molecular mimickery, role of gut >50% have ileal disease microscopic, breakdown of gut blood barrier can be inciting event of SpA) Inflammation (Most important is Enthesis, typical Sacroiliac joint, vertebral bodies, peripheral joint synovium, GI and eye, limited histopathology. Starts with early sacroilitis, then synovitis with myxoid bone marrow then destroyed bone replaced and endochondral ossification results in bone ankylosis) |
| How are clinical features of SpA? | Inflammatory back pain (IBP, first manifestation 75% of axSpA, lumbosacral junction, inactivity aggrevates and NSAIDs relieve, prolonged morning stiffness (>30min), nocturnal pain wakening, sacroilitis/spondylitis dx by expert criteria 4/5 improve w/exercise, pain at night, insidious onset, age <40, no improvement with rest) Ankylosis (Ossification of ligaments, costovertebral, sternocostal joints. Loss of lumbar lordosis, thoracic hyperkyphosis, forward stooping of neck, restricted spinal movement muscle spasms, respiratory failure decreased vital capacity and increased residual if hip involved we have bending forward) Osteoporosis fracture (spinal osteoporosis increase risk of fracture with lack of mobility and inflammatory cytokines, IBD higher risk) Peripheral Arthritis (Assymetric, oligoarticular, lower limbs, mechanical microtrauma, upper limb if PsA bilateral symmetrical polyarticular presentation. DIP joints see dactylitis sausage fingers)) Dactylitis (Very characteristic, ReA, PsA and undiff., sausage fingers/toes due to synovitis, enthesis, tenosynovitis, soft tissue swelling) Anterior chest wall pain (axial, 15% of pt, stero/clav/manubr, reduced chest expansion, can involve hips more than shoulders see hip pain, early bilateral for juvenile destruction and disability |
| How is enthesis clinical manifestation of SpA? | DIstinguishing feature, most typical is heel pain (posterior/inferior), in morning as soon as pt sets foot on floor and improves with ambulation, can be disabling and resist std anti-rheumatic tx, typically at achilles tendon plantar pain/ fasculitis Number of swollen joints is 44, 0-44 grading |
| What are comorbidites associated with SpA? | Cardiovascular (atherosclerosis), Osteoporosis/fracture, and MR 2 fold increased |
| What are lab findings in SpA? | No specific lab tests Seronegative disease ESR and CRP elevation (in only 40% of the cases CRP Elevation -> structural progression, CRP predictor of good response to treatment) HLAB27 (Found in 90% of patients with SPA (not in Lebanon) 8% in healthy subjects Not diagnostic value) |
| What are conventional image findings in SpA? | Conventional Radio (not helpful early, structural changes reflect changes by inflammation not inflammation itself, we may get radiographic sacroilitis [blurring of cortical margins of subchondral bone, erosions and sclerosis, joint space appears wider than fibrous, bony ankylosis] X ray has a New York grading system [ I sus, II evidence of erosion/sclerosis, III erosions and early ankylosis, IV total ankylosis, sensitivtiy first year 0%, after 10 years 60%, in spine we see squaring of vertebra secondary to erosions, vertebral enthesitis may cause sclerosis of upper and lower vertebra appear shiny corners romanus sign, syndesmophyte formation, bamboo spine |
| What are MRI findings in SpA? | Early detection, Axial SpA ASAS criteria include inflammation in SI joints seen on MRI, It can produce excellent evidence of sacroilitis and enthesitis. T2 weighted fat suppressed fast spin-echo and short Tao inversion recovery (STIR) sequences. Assessment of bone marrow edema (BMO) represents a sign of inflammation. Positive MRI put by ASAS/OMERACT MRI working group (active inflammation lesion of SI, BMO/osteitis clear located in typical anatomical sites (subchondral/periarticular), solitary BMO there should be 2 consecutive slices, if more BMO lesions on one slice, if we see inflammation it is sufficient, presence of inflammation alone is not enough |
| What is axial SPA/ AS? | Spinal pain resulting in limitation of spinal mobility, radiologic evidence of structural changes in SI joints and spine, enthesitis (40-60%) acute anterior uveitits (30-50%), disease course highly variable (axial inflammation + restricted spine mobility and decreased function), starts early adulthood, lifetime impact of stiffness, fatigue, limitation of social activities and participation |
| What are the new york criteria for AS? | Diagnosis (Clinical [low back pain and stiffness for more than 3 months improves with exercise not relieved by rest, limitation of spinal motion both saggital and frontal plane, and limitation of chest expansion relative to normal values] Radio [Sacroilitis grade >2 bilaterally/ grade 3-4 unilaterally]) Grading (definite dx if radio finding + 1 clinical, probable if three clinical , or radio alone) |
| What are signs and symptoms of AS? | Peripheral manifestations (enthesitis, peripheral arthritis, dactylitis) Extra-articular (anterior uveitis, subclinical inflammation of gut, lung abnormalities, bone abnormalities, renal, cardiac, psoriasis, and IBD.) |
| How is ASAS criteria for peripheral SpA? | Peripheral Arthritis/ Enthesitis/ Dactylitis + either >1SpA feature [uveitis, psoriasis, Crohn's, infection, HLAB27, Sacroilitis] or >2 SpA feature. |
| What is Psoriatic Arthritis? | Prevalence among caucasians 1-3%, female=male, 10-30% have PsA and psoriasis (Skin before most, simultaneous 20% and arthritis before 15%), arthritis onset 30-50 years, no correlation between skin and joints Manifestations (peripheral assymetrical oligoarticular, polyarticular symmetrical, assymetrical with DIP involvement and dactylitis, predominant spondylitis 5%, arthritis mutilans (pencil in cup on Xray) Juxta-articular new bone formation, absence of peri-articular osteopenia, and preservation of the joint space. We have nail involvement |
| What is SAPHO classification of PsA? What is caspar classification? | Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis. Dermato entity (acne and pustulosis), synovitis of anterior chest wall and SI joint, spondylodiscitis, enthesopathy, aseptic osteomyelitis / hyperostosis. 7 criteria, more than 3 points gives PsA. |
| What is enterohepatic arthritis? | Occurrence of inflammatory arthritis in patients with IBD UC or CD The prevalence of arthritis 17% to 20%, with a higher prevalence in Crohn’s disease Peripheral arthritis (Oligoarticular , usually transient, migratory and non-deforming generally self-limiting, but recurrences) Axial involvement and enthesitis may become chronic and destructive. Intestinal symptoms usually before with or after joint involvement |
| What is reactive arthritis? | Aseptic arthritis occurs 1 month of a primary infection, acute, assymetric oligoarthritis Extra-articular (ocular inflammation, enthesitis/mucocutaneous lesions [Balanitis circinata ring shaped skin on glans of penis/ keratoderma blennorhagia], Urethritis, Rarely Carditis) Duration 4-5 months of arthritis, recurrent attacks, chronic/recurrent peripheral arthritis, sacroilitis, or spondylitis. |
| What is sarcoidosis? | Multisystem inflammatory disorder, unknown etiology, young adults 20-30, epithelioid non-caseating granuloma, hetergeneous severity, Lungs affected in 90% of pt which is major cause of M&M, typically with one or more of (bilateral hilar adenopathy, pulmonary infiltrates, skin/eye lesions, joint manifestations |
| How is pathogenesis of sarcoidosis? | Epithelioid non-caseating granuloma formation, any tissue could be affected, Th1 CD4+ T cells, macrophages express cytokines and TNF, granuloma can either resolve w/out sequelae, or undergo obliterative fibrosis with interstitial firbosis. Granuloma is formed of border of CD8+ T cells, monocytes, mast cells, fibroblasts, and center of macrophage, epithelioid cells, Multinucleated giant cells and CD4+ T cells |
| What is the etiology causing sarcoidosis? | Infection, genetics, CD4+ T cell, Cytokines (IL2, IL12, IL18, IFN gamma, TNF alpha) |
| What are pulmonary clinical manifestations of sarcoidosis? | Pulmonary sarcoidosis (90%, cough, dyspnea, chest pain, usually self-limited goes to remission, degree of pulmonary invovlement affects remission rate, bilateral hilar adenopathy Stage I 50% (75% regress w/in 3 years and 10% stay chronic enlarged), Bilateral adenopathy + pulmonary infiltrates Stage II 25% (66% spontaneous remission and 33% progresses or remains unchanged, have mild to moderate symptoms) Stage III (10% spontaneous remission 14%, dense pulmonary infiltrates w/out adenopathy) Stage IV (pulmonary fibrosis) |
| What are extrapulmonary clinical manifestations of sarcoidosis (Musculoskeletal and Dermatologic)? | Musculoskeletal (4-38%, arthropathy [oligoarthritis, good prognosis in case of Loefgren syndrome, rarely chronic or deforming, dactylitis and tenosynovitis], Bone lesions [cystic, lytic, sclerotic, focal, osteopenia/osteoporosis], muscular disease, vasculitis) Loefgren syndrome [triad hilar adenopathy, acute arthritis/arthralgia often ankles bilateral, erythema nodosum. Usually self-limiting and good prognosis) Dermatological (30%, maculopapular eruptions [nose, lips, eyelids, forehead, back of neck], nodular lesions [face, trunk, extensors of limbs], lupus pernio [violent discoloration of nose/cheeks], erythema nodosum [panniculitis], atypical [ulcer, psoriasis, hypopigmentation.]) |
| What are extrapulmonary manifestations of sarcoidosis (Opthalmic, neurological and others)? | Ophthalmic (anterior uveitis, posterior uveitis, retinal vasculitis, keratoconjuctivitis, scleritis (very rare), conjuctival follicles) Neurological (5% of pt, females mainly, early in disease, life threat, agranulomatous meningitis with infiltrates and compression, cognitive or psychic manifestations (most common, seizures, headache, hypothalamic, hypopituotarism, DI, hydrocephalus, facial palsy) periphearl neuropathy, correlated with cardiac invovlement) Reticuloendothelial, GI, Reproductive, endocrine and exocrine glands [parotid in Heerfordt's syndrome, r/o sjogren], renal and electrolyte [Ca metabolism abnormality due to hyperproduction of Vit D extrarenal], Cardiac disease [most serious, infiltrate of ventricular septum, cause arrhythmias, heart block, sudden death, palpitations, syncope, dizziness, chest pain, see cardiac PET/MRI) |
| What are labs done for sarcoidosis? | Anemia of chronic disease, Leukopenia [lymphopenia 40% of pt], ESR, hypercalciuria 50% and hypercalcemia10-20%, hypergammaglobinemia 30-80%, serum ALP, ABG. Serum ACE (elevated 40-90% of untreated pt w/chronic sarcoidosis, produced by epithelioid and macrophages w/in granuloma, ACE monitor assesses course of disease unclear. |
| What are image findings in sarcoidosis? | ▪ Chest xray (check before) ▪ High resolution CT scan of the chest (Hilar and mediastinal lymphadenopathy, Nodules, Bronchial wall thickening , Ground glass phenomenon , Parenchymal masses or bands, Cysts, Fibrosis ▪ Bone xray ▪ PET scan ▪ Pulmonary function tests ▪ Bronchoscopy with bronchoalveolar lavage (Lymphocytes CD4+>CD8+) |
| How is dx of sarcoidosis done? | ▪ History ▪ Physical examination ▪ Posteroanterior chest radiograph ▪ Electrocardiography ▪ Pulmonary function tests ▪ Peripheral blood counts, ESR , Serum chemistries, including creatinine, calcium, and liver enzymes ▪ Routine ophthalmologic examination ▪ Tuberculin skin test ▪ Urine analysis, 24 hour urinary calcium ▪ Diagnostic procedures ▪ Tissue biopsy |
| How is course of sarcoidosis? | Often benign with good prognosis, spontaneous remission 60-70% of cases. Factors of bad prognosis (black race, age >40, lupus pernio, chronic uveitis, chronic hypercalicemia, nephrocalcinosis, progressive pulmonary involvement, neurosarcoidosis, cardiac involvement, nasal mucosal involvement, cystic bone lesions) |
| What are crystal diseases? | Monosodium Urate (MSU) and Gout. Calcium pyrophosphate (CPD) and pseudogout. Basic Calcium phosphate (BCP) and periarthritis Ochronosis. |
| What is gout? | MSU crystal deposition disease, elevated serum urate acid SUA causing hyperuriciemia leading to local conditions. We first get elevated purines (due to catabolism, tumor lysis syndrome, diet [meat, sea food...]) which lead to increased Xanthine and thus by xanthine oxidase increase urate pool causing hyperuricemia (excreted 30% fecal and 70% urine) causing (joint inflammation, kidney/bladder stones, nephropathy, CVD, metabolic syndrome as we get decreased renal clearance by drugs, fructose, genetics and kidney disease aggravating gout Risk factors (male, age, obesity, ethnicity pacific, polymorphism, kidney disease) |
| How is gout progression? | Starts asymptomatic, until we get first acute flare, then a period of years of no symptoms to have a second flare, then periods of no symptoms (intercritical perioids getting shorter) and flares which are more intense (Recurrent acute gouty arthritis), and finally we get to start of advanced gout |
| How is etiology of gout? | Depends on age and gender (very low prepubertal and adult women, high post menopausal women and postpubertal men) 3 factors influence hyperuricemia (total amount of produced uric acid, renal clearance of urate, and intestinal excretion of urate) |
| How is hyperproduction and decreased clearance of gout and local conditions causing gout? | Hyperproduction (increased purines [tumors, hematologic disease, chemo, psoriasis, food and beer], enzymatic defects [Lesch-Nyhan in boys, G6PD]) Decreased clearance (most common cause of gout, CKD, metabolic syndrome, HTN, decompensated HF, saturnine gout, alcohol, drugs [diuretics, aspirin, cyclosporine], genetics) Local conditions (Preference of lower limb [due to repititive microtrauma], osteoarthritis, cold temperature, severe intercurrent disease [hypoxia of tissue], surgery, uricosic drugs, some food? |
| How is pathophysiology of gout? | Formation of MSU crystals (hyperuricemia, precipitation of MCU crystals and deposition in articular and periarticular tissues) Acute gout attack (phagocytosis of the crystals, cell swelling and inflammation, cytokine production and vasodilation leading to neutrophil and monocyte influx) Chronic tophaceous gout (neutrophil death by NETosis, packaging of MSU crystals , inactivation of cytokines and resolution of inflammation) Normal uric acid is 6-6.6 mg/dl in serum, they accumulate in blood and tissues, precipitate in suprasaturated tissues form needle shaped crystals, they are less soluble under acid and low temperature conditions (cool peripheral joints). Urate crystals are recognized by polarizing microscopy by light retarding characteristic of urate. |
| How is physiopathologic cycle of gout? | Intercritical gout is quiecent, then comes urate crystal recognition by phagocytes, that release inflammasomes (IL1beta, IL6, IL18, TNF alpha, IL10) then amplification phase due to tissue damage, matric metalloproteinases, PGs, TLRs, then comes resolution phase (anti-inflammatory mediators like CD68, PPAR, TGF) |
| How is epidemiology of gout? | No stats, worldwide distribution, regional differences reflect environmental dietary and genetic differences. Prevalence is 5.9% men and 2% women (difference due to age of onset, estrogen has uricosic effect so gout is unusual in premenopausal women) Western countries affected 1-2% w/ prevalence with age 7% men >65 and 3% women over 85 uric acid elevated 10-20 years prior to gout. |
| How is clinical presentation of gout? | Typical presentation (monoarthritis, acute excrutiating pain, edema, inflammation of MTP of great toe (podagra initial manifestation 50% of cases, and 90% involved) Most common sites (ankle, wrist, finger, knee), early gout 1 or 2 joints involved, any pt with acute monoarthritis it is sus. Polyarticular is in 10% of pt, usually consequetive joints, reach max intensity w/in 8-12 hours, affected joints hot, red, tender, swollen bed sheet, if untreated it resolves w/in 2 weeks |
| How is physical exam of gout? | All joints examined, involved joints signs of inflammation, erythema over joint looks like cellulitis, skin desquamate as the attack subsides, joint capsule quickly swollen loss in ROM, fever, differential with septic arthritis or coexisting acute gouty arthritis, commonly involves small joints of fingers and toes and kness and can cause inflammation in synovial bursae/tendons. Tophi (collections of urate crystals in soft tissues, develop after a decade of chronic untreated gout, earlier in older women those recieving diuretics, classical located along ear helix, fingers, toes, prepatellar bursa, olecranon. DD rheumatoid nodule. |
| How is complication of gout? | • Severe degenerative arthritis • Secondary infections • Increased susceptibility to infection • Urate or uric acid nephropathy • Renal stones • Nerve or spinal cord impingement • Fractures in joints with tophaceous gout |
| How is gout prognosis? | Good if treated early, considerable morbidity, acute episodes are handicapping, chronic injury to intra-articular cartilage leaves joints susceptible to joint infections, untreated tophi can lead to severe joint destruction, renal impairment, spinal cord impingement when deposition in tissues produces a large mass. Hyperuricemia and gout are associated with increased mortality Association with heart and vascular diseases, renal diseases, metabolic disordes, and joint diseases |
| How is gout differential diagnosis? | Septic Arthritis,Cellulitis, Pseudogout, Rheumatoid Arthritis, Arthritis as a Manifestation of Systemic Disease. Characteristics for gout (male, previous arthritis attack, onset w/in 1 day, joint redness, first MTP, HTN or cardiovascular disease, serum uric acid level higher than 5.88 mg/dl |
| How is dx of gout occurring? | Joint aspiration (Demonstration of intracellular monosodium urate crystals needle-shaped intracellular and extracellular crystals Negatively birefringent urate crystals are seen on polarizing examination in 85% of specimens Sensitivity 84%,specificity 100%) Exclusion of infection or other crystal types in synovial fluid from inflamed joint |
| What is the work up for gout? | Blood test (SUA [could be normal during attack hx of high level], inflammatory markers, kidney function test) Renal uric acid measure in high risk pt (renal calculi, strong fam hx, first attack before 25) Xray Erosions (overhanging edges, bony erosions, punched out border [cookie cutter], asymmetrical, predilection of distal joints) US (double contour sign, hyperechoic, irregular line of crystal on surface of cartilage, wet clump of sugar tophi hyper and hypoechoic, bony erosions adjacent to tophi deposits) |
| What is calcium pyrophosphate deposition disease? | Metabolic arthropathy caused by deposition of CPD in and around joints especially articular and fibrocartilage, often asymptomatic, with only radio changes seen, various clinical manifestations may occur include acute (pseudogout) and chronic arthritis. Almost any joint involved mostly knees, wrists and hips, it is most common cause of secondary metabolic osteoarthritis. Five common presentations (asymptomatic [lanthanic] CPPD, acute pseudogout, pseudoosteoarthritis, pseudorheumatoid arthritis, pseudoneuropathic joints) Prognosis (significant morbidity due to acute attack pain, tx is important to prevent further end-organ damage but cannot reverse the joint disease) |
| How is etiology of CPPD? | Unknown, increased ATP breakdown w/resultant increased inorganic PP in joints resulting from aging, genetic factors, or both. Changes in cartilage matrix may play an important role promoting CPPD, rare heriditary form of CPPD occur auto dominant. |
| How is epidemiology of CPPD? | 50% people older than 85 years have chondrocalcinosis, sex and age related demographic (more in women than men slightly, if early associated with secondary cause like metabolic syndrome/familial cause) Comorbidities (HyperPTH, gout, osteoarthritis, RA, hemochromatosis, osteoporosis, hypoMg, CKD, calcium supplementation |
| How is clinical presentation of CPPD? | Asymptomatic (Lanthanic, radio chondrocalcinosis most common form, hyaline cartilage and fibrocartilage of knees, triangular ligament of wrist , of pubis symphysis, acetabulum) Acute Pseudogout (acute monoarticular/oligoarticular arthritis, 25% of cases, acute monoarthritis with pain and swelling, olyarticular may occur on occasion, could be precipitated by MI, CHF, stroke, surgery, trauma increasing calcium level) Pseudo-osteoarthritis (MCP, wrists, elbows, shoulders unlikely, knees, spine occur in primary osteo-arthritis, 50% of all cases and half are pseudogout) PseudoRA (5% of pt, symmetrical inflammation of PIP and MCP morning stiffness and joint swelling) Pseudoneuropathic joints (<5% of pt, severe destructive arthropathy, no clear neuro disorder present, chondrocalcinosis on Xray_ |
| How is physical exam of CPPD? | Vary depending on the form of CPDD Acute pseudogout( an acutely inflamed joint with swelling, effusion, warmth, tenderness, and pain on range of motion typically occurs in the knee but may be present in the wrists, shoulders, ankles, hands, and feet.) Pseudo-osteoarthritis (Osteoarthritis like, sometimes with an unusual joint predilection If a patient has osteoarthritis involving the MCP joints and wrists, consider CPPD associated with an underlying metabolic disease.) Pseudorheumatoid arthritis (rheumatoid arthritis with synovitis in a symmetrical, polyarticular pattern, especially involving the wrists and MCP joints.) |
| What are complications of CPPD? | Acute synovitis and degenerative arthtitis, joint destruction from neuropathic like arthropathy (rare), sometime invasive CPPD, coexistent infection, Crowned dens syndrome |
| How is dx of CPPD done? | Arthrocentesis (see calcium pyrophosphate in tissue/synovium, no positive birefringence by compensated polarized LM) X ray (radio calcifications in knees, wrist, pubic symphysis and shoulders) US (cartilagenous calcifications) |
| How is workup of CPPD? | WBC and ESR elevated, underlying metabolic disease, get serum Ca, P, Mg, ALP, iron workup, pseudogout (leukocytosis with left shift), crystals appear rhomboid difficult to see, If intracellular acute pseudogout may suggest it, aspiration we see WBC 10,000-50,000 90% neutrophils) Radio (Hooklike osteophytes seen for hemochormatosis, pseudosteoarthritis and simple CPPD, US shows difference between gout and chondrolithiasis |
| What is the difference between gout and pseudo gout? | Crystal composition (Uric acid vs Calcium pyrophosphate) Crystal shape (needle-like vs rhomboid) Birefringent (negative vs weakly positive) Most common affect 1st joint (MTP vs knee) Radio (rat-bite erosion vs white lines of chondrolithiasis) First line tx (both NSAIDs) |
| What is Ochronosis? | Bluish black discoloration of tissues, seen with alkaptonuria (rare autorecessive disorder of deficiency of homogentisic acid oxidase, so we get accumulation of homogentisic acid in tissues, involvement of pigmentation of chorndrocytes by IL6, tissues weakly brittle lead to chronic inflammation, black urine. |
| What is BCP crystal disease? | Musculoskeletal syndromes two categories (arthritis associated with BCP crystal [osteoarthritis] and calcific periarthritis) Predominant mineral type seen is carbonated hydroxyapatite. BCP crystals (similar in composition to both normal minerals found in bone and teeth, and pathologic minerals found in atherosclerotic plaques and calcinosis cutis) |
| How is pathogenesis and risk factors for BCP crystal diseases? | Not fully understood, occur in sites of injury, produce symptoms and mediate tissue damage by several mechanisms including induction of inflammation, biochemical disruption, interaction with CT, producing cytokines in absence of inflammation. Risk factors include (severe radio damage in OA, CPP crystals, hx of trauma/heavy use [Milwaukee shoulder syndrome], ESRD, CT disease, genetic) Average age is 72 years. |
| How is clinical presentation of BCP? | Osteoarthritis (similar to typical OA, rapid progression to end-stage disease) Milwaukee shoudler syndrome (chronic syndrome of shoulder arthritis, loss of function and shoulder pain, older adult pt, especially women, large but non-inflammatory shoulder effusions) Calcific periarthritis (acute severe pain in single joint/tendon/bursa, large and small joints and spine, may have fever, healthy and middle-aged people, women predominate, shoulder 2.7%, joints appear tender, warm, swollen and erythematous with no joint effusion), self limited attacks weeks to months) |
| How is the different presentation of calcific periarthritis in different joint types? | Large joints (shoulder [ gradual shoulder pain no trauma, pain top or lateral of shoulder radiate to deltoid insertion, increased pain at night inable to move affected shoulder] Hip [rectus femoris pain ad tenderness AIIS, exacerbated by hip flexion, snapping hip syndrome calcific deposits to rectus femorus, glut max pain proximal thigh and glut min pain low back]) Small joints (hand flexor carpi ulnaris, maybe hand tendons, foot MTP1 and symptoms plantar aspect) Other sites (retropharyngeal in cervical spine onset of neck pain and stiffness with dysphagia and odynophagia) |
| How is physical exam of BCP? | Glenohumeral joint effusion, crepitation, and joint instability Routine laboratory studies are generally normal/ Synovial fluid analysis( Alizarin red S staining for calcium-containing crystals can identify calcium-containing crystals in synovial fluid with ordinary light microscope, orange-red clumps, neither sensitive nor specific for BCP crystals) |
| What are image findings in BCP? | X ray (MSS (Involved shoulder joints, severe joint destruction, cartilage loss, rotator cuff damage) Periarthritis (extraarticular calcifications near tendons)) US (hyperechoic lesions with acoustic shadow, In the resorptive phase the deposit may appear more fluffy, fragmented, or punctuated) |
| What is SLE? | Chronic autoimmune inflammatory multisystem disorder, with waxing and waning course, significant morbidity, diverse clinical manifestations and variable course and outcome. Prototype of autoimmune disease, loss of tolerance to self leads to immune system mediated damage, unknown etiology, expresses itself differently in each individual |
| How is natural history of SLE? | A combination fo genes and environment play a role, gets to preclinical setting where we get autoantibodies general/specific, then we get to clinical phase with inflammation and involvement of first organs, then get flares, additional organs, damage and comorbidities increase the course of action (infections, atherosclerosis and malignancies) |
| How is epidemiology of SLE? | WW, radial variation (black, chinese and asian more common), prevalence 12-50/10000, female predominance 9 to 1 (increases during child bearing age 30 to 1. Genetics (familial aggregation [5-12%, concordance increase risk in monozygotic twins to 24-70% and dizygotic to 2-5%] HLA II DR2 and DR3 involved, inheritied complement deficiencies involved (C1q, C2, C4), some may influence clinical manifestations) Environmental (Physical [UV, thermal burns], chemical [drug], infectious [EBV], hormonal [estrogens], and autoantiboides) |
| How is pathogenesis of SLE? | Apoptosis (abnormal, source of autoantigen, UV induced in skin and impaired clearance) Nucleic acids (target in SLE linked to apoptosis, loss of T cell tolerance and self-recognition) Innate immunity (TLRs and inflammasomes on cell membranes activate Dc, Dc autophagy occurs and regulates IFNa prodcution and makes APCs to CD4+ T cells, IFNa activates autoantibodies specific, complement clearance of apoptotic material, Neutrophils promote inflammation, and endothelia impaired repair due to DNA degradation) Adaptive immunity (T and B cells activated polyclonal B cells form autoantibodies, B lymphocyte stimulator soluble TNF for B cell survival and differentiation, Immune complexes impaired clearance cause tissue injury at sites like skin and kidney) |
| How is SLE disease mechanism and tissue damage? | Mediate by inflammatory cells, ROS, cytokine production, and modulation of coagulation cascades. Deposition in tissues and vessels, cell mediated cytotoxicity, autoantiboides, and proinflammatory cytokines. |
| How is SLE clinical manifestation of skin? | Acute rashes, malar rash (butterfly rash, transient or permanent rash non scarring, erythematous raised lesion pruritic or painful, malar distribution photosensitive), subacute rashes, subacute cutaneous LE (10% of SLE pt, photosensitive, begin as small erythematous lesion in back), chronic rashes, discoid lupus erythematous (DLE, up to 25% of pt, discrete erythematous slightly infiltrated plaques, active inflammation at periphery and heal leave a scar or dyspigmentation, on face neck and scalp Others (lupus profundus, lupus tumidus, livedo reticularis, vasculitis, periungeal erythema, alopecia [scarring or lupus hair]) Photosensitivity (inflammatory rash after UV exposure, from sunlight or fluorescent light, lasts days to weeks, 60-100% of pt with SLE) |
| How is SLE clinical manifestation in mucus membranes and musculoskeletal system? | Mucus membranes (25-45% of pt, ulcers, painless, mouth>nasal) Musculoskeletal (53-95% of pt, arthritis (non-erosive, non-deforming arthritis, transient migratory or reversible, rhupis, Jaccoud arthropathy [hand deformity MCP joints/swan neck]) Myositis (generalized, inflammatory 4-16%) avascular bone necrosis (major cause of morbidity and disability, shoulders hips and knees, induced ischemia by raynauds vasculitis, fat emboli, cortisol, Antiphospholipid syndrome (APS), osteonecrosis shortly after shot of high dose corticosteroids) |
| How is SLE renal clinical manifestations? | 40-50% of pt, major cause of morbidity and hospitalization, immune complexes formation in kidney, proteinuria, dysmorphic erythrocytes, to be detected important to have urine analysis sees kidney function) Classification of Lupus nephritis by WHO: Class I minimal mesangial involvement, Class II mesangial, Class III focal glomerulonephritis, Class IV diffuse glomerulonephritis, Class V membraneous nephritis, Class VI advanced sclerosing |
| How is SLE neuropsychiatric clinical manifestations? | Common 25-50% of pt, CNS see any neuro disorder, PNS any neuropathy |
| How is SLE cardiovascular clinical manifestation? | Pericarditis 12-25% of pt, pericardial effusion may be asymptomatic mild to moderate, increased M&M from CVD by accelerated premature atherosclerosis and valvular heart disease (linked to APS), Libman-Sacks endocarditis. |
| How is SLE pleuropulmonary clinical manifestation? | Pleuritic chest pain common, pleural effusion exudate uni or bilateral, acute pneumonia uncommon with high MR, ILD, bronchiolitis obliterans with organising pneumonia required biopsy to dx and respnds to corticosteroids, capillaritis or diffuse alveolar hemorrhage rare due to APS, poor prognosis. Shrinking-lung syndrome, possible cause is diaphragmatic weakness in longstanding SLE, PE or infarct, Pulmonary HTN, infection, malignancy. |
| How is SLE lymphadenopathy and splenomegaly clinical manifestation? | •Lymphadenopathy occurs in about 40% of patients • usually at the onset of disease or during disease flares (Shapira et al, 1996). • more likely to have constitutional symptoms •A lymph node biopsy may be warranted when the degree of lymphadenopathy is out of proportion to the activity of the lupus •Splenomegaly occurs in 10–45% of patients, particularly during active disease |
| How is SLE hematologic clinical manifestations? | Anemia (common, chronic disease, hemolysis, blood loss, renal insufficiency drugs, infections, hypersplenism, myelodysplasia, aplastic anemia) Leukopenia (WBC <4500 reported 30-40% of pt, lymphocytopenia in 20% of pt <1500) Thrombocytopenia (mild in 25-50% of pt, immune mediated platelet destruction, microangio hemolytic anemia or hypersplenism, drug induced) |
| How is SLE GI clinical manifestations? | GI manifestations are reported in 25–40% of patients with SLE, and represent either lupus GI involvement or effects of drug treatment (Ebert and Hagspiel, 2011). Dyspepsia peptic ulcer Acute abdomen Pancreatitis Autoimmune hepatitis |
| How are criteria of dx for SLE? | We have clinical and immunologic criteria, required >4 or biopsy ANAs (seen by immunofluorescence, low specificity, in healthy/low titer individuals) Ab to extractable nuclear Ag (anti-dsDNA [LN end stage renal disease increased severity and poor survival], APS [venous, CNS, death], anti-Ro/La [neonatal lupus, congentical heart block, seropositive mothers and children) |
| How is prognosis, M&M of SLE? | Prognosis dramatic improvement when managed but long term. M&M (infections [after immune regulation], atherosclerosis, osteoporosis, malignancies) |
| What is systemic sclerosis? | Chronic multisystem autoimmune disease of CT, characterized by fibrosis of skin and internal organs, vasculopathy (microvasculature and humoral immunity) and part of scleroderma spectrum diseases (localized [morphea, linear scleroderma], or systemic [limited and diffuse]) |
| How is the classification of systemic sclerosis? | Limited cutaneous systemic sclerosis (lcSSc, skin involved only distal to knees and elbows) Diffuse cutaneous systemic sclerosis (dcSSc, skin thickening proximal to knees and elbows) SSc sine scleroderma (vascular and serological features without skin involvement) EUSTAR (Vedoss very early dx of SSc, three red flags (Raynaud's phenomenon, puffy fingers and ANAs plus 1 of (disease-specific autoantibodies [ACA/anti-topo], or microvascular alterations detected by nailfold videocapillaroscopy) |
| How is epidemiology of SSc? | Orphan disease, mainly underestimated |
| How is etiology of SSc? | MHC II, non MHC genes (associated with metabolism of ECM, and coding for proteins involved in innate immunity, macrophage activation and T cell function) Environmental factors (viruses [CMV, parvo, EBV], induce vascular damage and fibroblast proliferation, drugs, organic solvents [vinyl chloride, silica, nanoparticles]) |