FN: Sarcoidosis Flashcards Preview

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Flashcards in FN: Sarcoidosis Deck (23):
1

Definition

Multisystem granulomatous disorder of unknown cause

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Epidemiology

􏰀 Age: 20-40yrs
􏰀 Sex: F>M
􏰀 Geo: Afro Caribbean
􏰀 HLA-DRB1 and DQB1 alleles

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Clinical features pneuomic

GRAULOMAS
􏰀 In 20-40% the disease presents incidentally on CXR
􏰀

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acute sarcoidosis features

Acute sarcoidosis: EN, fever, polyarthralgia, BHL

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Pneumonic

General
Respiratory
Arthralgia
Neurological
Urine
Low Hormones
Ophthamology
Myocardial
Abdominal
Skin

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General features

􏰀 Fever
􏰀 Anorexia and ↓ wt.
􏰀 Fatigue
􏰀 Lymphadenopathy and HSM

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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

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Arthralgia features

􏰀 Polyarthralgia 􏰀 Dactylitis

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Neurological features

􏰀 Peripheral and cranial polyneuropathy (esp. Bell’s palsy)
􏰀 Meningitis, transverse myelitis, SOL

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Urine features

􏰀 ↑Ca → Renal stones, nephrocalcinosis, DI

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Low hormones features

􏰀 Pituitary dysfunction: e.g. amenorrhoea

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Opthalmological

􏰀 Uveitis
􏰀 Keratoconjunctivitis
􏰀 Sicca / Mikulicz / Sjogrens syndrome

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Myocardial features

􏰀 Restrictive cardiomyopathy 2O to granulomas + fibrosis
􏰀 Pericardial effusion

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Abdominal features

􏰀 Hepatomegaly + cholestatic LFTs 􏰀 Splenomegaly

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Skin features

􏰀 􏰀
EN: painful erythematous nodules on shins (paniculitis) Lupus pernio: raised, dusky purple plaque on nose, cheek, fingers

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Ix Bloods show

􏰀 ↑ESR
􏰀 ↑Ca
􏰀 Lymphopenia 􏰀 ↑seACE
􏰀 ↑Ig
􏰀 ↑LFTs

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Tuberculin skin test shows

􏰀 Negative in 2/3

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Lung function tests

􏰀 Restrictive pattern ̄c ↓FVC
􏰀 ↓ transfer factor

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Tissue biopsy

􏰀 Lung, LNs, skin nodules, liver
􏰀 Diagnostic: non-caseating granulomas

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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

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Prognosis

􏰀 60% ̄c thoracic sarcoidosis resolve over 2yrs
􏰀 20% respond to steroids
􏰀 20% no improvement despite Rx

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BHL Differential

􏰀 Sarcoidosis
􏰀 Infection: TB, mycoplasma
􏰀 Malignancy: lymphoma, carcinoma 􏰀 Interstitial disease: EAA, silicosis

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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

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