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Sarcoidosis
Rheumatology
| Question | Answer |
|---|---|
| Sarcoid: classic triad of sx | hilar adenopathy, erythema nodosum, and arthritis |
| Sarcoid arthritis: | usu symmetric & migratory; often in the ankles |
| Sarcoid joint effusions are usually: | non-inflammatory |
| Sarcoid arthritis is: | nondeforming, nonerosive, & self-limiting, usually not lasting more than 3-4 months |
| Defn sarcoidosis | noncaseating granulomatous multisystem dz; lungs, lymph nodes, skin, liver, eyes; 2nd & 3rd decade |
| Sarcoid prevalence | Swedes, Danes, AA; F>M |
| Sarcoid lifetime risk | 0.85% (W); 2.4% (AA) |
| Sarcoid mortality | d/t resp fail = 1-5% |
| Sarcoid Diathesis | Ethnicity, hormones, age, various immune complexes, and environmental factors. |
| Granuloma is composed of: | Macrophages (epithelioid cells ); Multinucleated giant cells; Lymphocytes |
| 3 stages of sarcoid granuloma formation | Trigger CD4+ Th1 cells by local APCs; Release of cytokines with multiple / overlapping fns; Accumulation of immunocompetent cells at sites of ongoing inflammation |
| Sarcoid granuloma 3 stages: see = | periph ring of lymphocytes; giant cells; hyalinization & fibrosis |
| Sarcoid: chronic arthropathy = | Synovial thickening and effusions |
| Acute sarcoid arthropathy: Tx | NSAIDs; occasionally, ST corticosteroids |
| Chronic sarcoid arthropathy: Tx | Depends on severity of extra-articular manifestations that usually accompany it; C’steroids are usu needed to ctrl systemic dz |
| Sarcoid pathophysiology | Ag / T cells; CD8 & T cells reg common stim factor, dendritic cells can’t turn it off; monos become macrophages, lead to granulomas; no mechm to turn process off, macrophages invade & destroy organs |
| Most common type of lesion in sarcoid | Maculopapular sarcoidosis |
| Erythema Nodosum = | red, hot, tender, shiny areas of the shins, calves, knees, & buttocks |
| Lupus pernio = | chronic persistent lesion w/ predilection for the nose, cheeks, and ears |
| Nail lesions, lupus pernio, and bone cyst most commonly seen in: | African Americans |
| Neurosarcoidosis | 4%- 7% of pts; CN lesions most common; Facial palsy (7th Cranial Nerve) most frequent presentation |
| Ocular Sarcoidosis involves: | both ant & post segments of the eye; ant uveitis more frequent (acute or chronic iridocyclitis); poss also keratoconjunctivitis sicca (chronic fibrotic longstanding sarcoid) |
| Acute sarcoid iridocyclitis | Abrupt onset; Pain, photophobia, blurred vision, reddening of the eyes |
| Chronic sarcoid iridocyclitis | Insidious onset; Gray, mutton fat-like keratic precipitates, prolonged course; fibrosis leads to cataracts or glaucoma; Pain, photophobia, blurred vision, red eyes |
| Pulmonary sarcoid: dx 3 ways | Chance (CXR); nonresp sx (skin, occular, heart failure); Acute resp sx (cough, CP, SOB on exertion, malaise, fever) |
| Sarcoid: most common affected organ = | lung (dx by CXR; bullus) |
| Stage 0 | Normal Radiograph, abnormal PFTs |
| Stage 1: | Bilateral Hilar Lymphadenopathy |
| Stage 2: | Bilateral Hilar Lymphadenopathy & Parenchymal Infiltration |
| Stage 3: | Pulmonary Infiltration with Hilar Lymphadenopathy |
| Stage 4: | Advanced Fibrosis, Bullae, Cysts |
| Incidence liver involvement in sarcoid | 90% of cases; epithelioid granulomas |
| Sarcoid MS involvement | most often small bones of hands / feet (sausage) |
| Sarcoid GI involvement | dilated esophagus (rare); distal third of stomach |
| 3 types of sarcoid bone lesions | permeative, lytic, obstructive (destructive) |
| Permeative lesion = | tunneling of cortex, then remodeling (hands get perm & lytic) |
| Lytic lesion = | Larger, round, punched-out cysts involving cortex / medulla of medial / proximal phalanges |
| Destructive bone lesion = | Rapid advancing bone involvement w/ multiple fx’ / devitalized cortex (feet get lytic & destructive) |
| Sarcoid Polyarthralgia assoc with: | erythema nodosum and bilateral lymphadenopathy |
| Polyarthralgia: 3 types: | Fitting polyarthralgia; Bone sarcoidosis; Synovial thickening |
| Sarcoid: MS: clin findings | Granulomatous myopathy; Mx weakness; Hypercalcemia; Acute (polymyositis) or chronic; mx biopsy: granulomas |
| Sarcoid: Dx | Bx; Bronchoscopy; PFT; serum ACE; CXR; Gallium Scan; MRI / CT; Vit D sensitivity; Mediastinoscopy |
| Sarcoid: Tx | Corticosteroids (prednisone); methotrexate; Plaquenil |
| Sarcoid prognosis: Acute | Stage 1 / 2: resolution in up to 46%; Erythema nodosum resolves in 1 mo; Bilat hilar LA in 1-2 mo |
| Sarcoid prognosis: Chronic | Fibrotic clinical / histo; Normal ACE; Multisystem Dz |
| Neurosarcoidosis: Common sites: | hypothalamus, third ventricle, pituitary gland |
| Neurosarcoidosis: on MRI, may resemble: | meningioma |
| Sarcoid: chronic arthropathy = | Synovial thickening and effusions |
| Lesions assoc w/ sarcoid occur in combination with: | intrathoracic dz, bone cyst, uveitis |
| Neurosarcoidosis | 4%- 7% of pts; CN lesions most common; Facial palsy (7th Cranial Nerve) most frequent presentation |
| Neurosarcoidosis: on MRI, may resemble: | meningioma |
| Erythema nodosum is assoc with: | Systemic autoimmune diseases (RA, IBD), OCP |
| Acute sarcoid arthropathy: Tx | NSAIDs; occasionally, ST corticosteroids |
Created by:
Abarnard
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