| 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) |
