Sarcoidosis

 Is a multisystem chronic non-caseating granulomatous disease of

unknown aetiology.
 Aetiology: still unknown.

Risk Factors:
 More common in women than men.
 Most common in young adults of African descent.
 More common in non-smokers.

Pathology:

 Non-caseating granuloma formation (lungs, liver, skin, eyes,

spleen, parotid gland..).
 Sarcoidosis causes anergy to common skin antigens that usually
elicit type 4 delayed immune response

Presentation:

 Asymptomatic.
 Respiratory symptoms (chronic dry cough,SOB).
 Constitutional symptoms (fever, malaise, anorexia and weight
loss).
 Extra-pulmonary symptoms: Erythema nodosum (painful nodules
on the anterior of the leg),
 Painless subcutaneous nodules.
 Lupus pernio: violaceous rash in nose and cheeks.
 Hypercalcemia.

Lofgren’s Syndrome
 Acute form of sarcoidosis characterised by:
 Erythema nodosum, uveitis, bilateral hilar lymphadenopathy
(BHL), Fever and polyarthropathy.
 Carries excellent prognosis.
Systemic Features:
 Anterior uveitis, Keratoconjunctivits sicca.
 Parotid gland enlargement and xerostomia.
 Lymphadenopathy and BHL.
 Cardiac sarcoidosis (restrictive cardiomyopathy) arrhythmia, Heart
block.
 Granulomatous liver disease, Splenomegaly.
 Hypercalcemia due to hypervitaminosis D, hypercalciuria and
kidney stones, increased ACE.
 Erythema nodosum (good prognosis).

Diagnosis:
 Lab tests: Hypercalcemia and hypercalciuria, high ESR.
 High ACE - High in active disease
 (Non-specific marker- can be used in disease monitoring).
 Tissue biopsy: Non-caseating granuloma.
 BAL: increased CD4:CD8 T-cell ratio.
 Spirometry: Restrictive lung disease.
 CXR:

0 Normal

1 BHL

2 BHL+ Infiltrates

3 Diffuse infiltrates only

4 Diffuse Fibrosis

TREATMENT:
 Self limiting: Spontaneous remission (majority).
 Acute illness and erythema nodosum (NSAIDs).
 Progressive disease: Glucocorticoids for progressive disease
(hypercalcemia, renal impairment...).