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Sarcoidosis

Rheumatology

QuestionAnswer
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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