FN: Sarcoidosis Flashcards

1
Q

Definition

A

Multisystem granulomatous disorder of unknown cause

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2
Q

Epidemiology

A

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

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3
Q

Clinical features pneuomic

A

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

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4
Q

acute sarcoidosis features

A

Acute sarcoidosis: EN, fever, polyarthralgia, BHL

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5
Q

Pneumonic

A
General
Respiratory
Arthralgia
Neurological
Urine
Low Hormones
Ophthamology
Myocardial
Abdominal
Skin
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6
Q

General features

A

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

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7
Q

Respiratory features

A

􏰀 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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8
Q

Arthralgia features

A

􏰀 Polyarthralgia 􏰀 Dactylitis

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9
Q

Neurological features

A

􏰀 Peripheral and cranial polyneuropathy (esp. Bell’s palsy)

􏰀 Meningitis, transverse myelitis, SOL

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10
Q

Urine features

A

􏰀 ↑Ca → Renal stones, nephrocalcinosis, DI

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11
Q

Low hormones features

A

􏰀 Pituitary dysfunction: e.g. amenorrhoea

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12
Q

Opthalmological

A

􏰀 Uveitis
􏰀 Keratoconjunctivitis
􏰀 Sicca / Mikulicz / Sjogrens syndrome

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13
Q

Myocardial features

A

􏰀 Restrictive cardiomyopathy 2O to granulomas + fibrosis

􏰀 Pericardial effusion

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14
Q

Abdominal features

A

􏰀 Hepatomegaly + cholestatic LFTs 􏰀 Splenomegaly

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15
Q

Skin features

A

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

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16
Q

Ix Bloods show

A
􏰀 ↑ESR
􏰀 ↑Ca
􏰀 Lymphopenia 􏰀 ↑seACE
􏰀 ↑Ig
􏰀 ↑LFTs
17
Q

Tuberculin skin test shows

A

􏰀 Negative in 2/3

18
Q

Lung function tests

A

􏰀 Restrictive pattern ̄c ↓FVC

􏰀 ↓ transfer factor

19
Q

Tissue biopsy

A

􏰀 Lung, LNs, skin nodules, liver

􏰀 Diagnostic: non-caseating granulomas

20
Q

Treatment acute vs chronic

A
􏰀 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
21
Q

Prognosis

A

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

22
Q

BHL Differential

A

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

23
Q

Granulomatous Disease Differential

A

􏰀 Infections: TB, leprosy, syphilis, crypto, schisto
􏰀 AI: PBC
􏰀 Vasculitis: GCA, PAN, Wegener’s, Takayasu’s
􏰀 Idiopathic: Crohns, Sarcoid
􏰀 Interstitial lung: EAA, silicosis