FN: Sarcoidosis Flashcards
Definition
Multisystem granulomatous disorder of unknown cause
Epidemiology
Age: 20-40yrs
Sex: F>M
Geo: Afro Caribbean
HLA-DRB1 and DQB1 alleles
Clinical features pneuomic
GRAULOMAS
In 20-40% the disease presents incidentally on CXR
acute sarcoidosis features
Acute sarcoidosis: EN, fever, polyarthralgia, BHL
Pneumonic
General Respiratory Arthralgia Neurological Urine Low Hormones Ophthamology Myocardial Abdominal Skin
General features
Fever
Anorexia and ↓ wt.
Fatigue
Lymphadenopathy and HSM
Respiratory features
Upper: otitis, sinusitis
Lower (seen in 90%)
Dry cough, SOB, chest pain, ↓ lung function
Stage 1: BHL
Stage 2: BHL + peripheral infiltrates
Stage 3: Peripheral infiltrates alone
Stage 4: Progressive mid-zone fibrosis ̄c bullae
Arthralgia features
Polyarthralgia Dactylitis
Neurological features
Peripheral and cranial polyneuropathy (esp. Bell’s palsy)
Meningitis, transverse myelitis, SOL
Urine features
↑Ca → Renal stones, nephrocalcinosis, DI
Low hormones features
Pituitary dysfunction: e.g. amenorrhoea
Opthalmological
Uveitis
Keratoconjunctivitis
Sicca / Mikulicz / Sjogrens syndrome
Myocardial features
Restrictive cardiomyopathy 2O to granulomas + fibrosis
Pericardial effusion
Abdominal features
Hepatomegaly + cholestatic LFTs Splenomegaly
Skin features
EN: painful erythematous nodules on shins (paniculitis) Lupus pernio: raised, dusky purple plaque on nose, cheek, fingers
Ix Bloods show
↑ESR ↑Ca Lymphopenia ↑seACE ↑Ig ↑LFTs
Tuberculin skin test shows
Negative in 2/3
Lung function tests
Restrictive pattern ̄c ↓FVC
↓ transfer factor
Tissue biopsy
Lung, LNs, skin nodules, liver
Diagnostic: non-caseating granulomas
Treatment acute vs chronic
Pts. ̄c asymptomatic BHL do not require Rx. Acute sarcoidosis Usually resolves spontaneously Bed rest and NSAIDs Chronic sarcoidosis Steroids: pred 40mg/d for 4-6wks Additional immunosuppression: methotrexate, ciclosporin, cyclophosphamide
Prognosis
60% ̄c thoracic sarcoidosis resolve over 2yrs
20% respond to steroids
20% no improvement despite Rx
BHL Differential
Sarcoidosis
Infection: TB, mycoplasma
Malignancy: lymphoma, carcinoma Interstitial disease: EAA, silicosis
Granulomatous Disease Differential
Infections: TB, leprosy, syphilis, crypto, schisto
AI: PBC
Vasculitis: GCA, PAN, Wegener’s, Takayasu’s
Idiopathic: Crohns, Sarcoid
Interstitial lung: EAA, silicosis