Sarcoidosis is a systemic inflammatory disease characterized by non-caseating granulomas of unknown cause but may be due to an altered immune response in genetically susceptible individuals after exposure to environmental or infectious agents. It commonly involves the lungs causing breathing problems, eyes causing uveitis, and skin causing lesions. Diagnosis is made by biopsy showing granulomas. Treatment involves steroids and immunosuppressants to reduce symptoms from organ involvement. Prognosis is variable but may include spontaneous remission or progression to respiratory failure.

1. Sarcoidosis
A systemic inflammatory disease
Characterized by non-caseating
granulomas

2. Pathophysiology
 Exact cause not known
 In genetically susceptible individuals possible
altered immune response after exposure to
environmental or infectious agent
(mycobacteria/Propionibacterium)
 Increased macrophage & CD4 T-helper cell
activation causes inflammation
 Suppressed IL-2 secretion causes anergy
 Macrophages convert vit. D to its active form

3. Clinical features
 Pulmonary- B/L hilar adenopathy,
obstructive/restrictive disease causes progressive
SOB & dry cough
 Eye- uveitis, granulomatous conjunctivitis,
optic neuritis/retinitis
 Skin- nodules/plaques over face/extensor surfaces of
limbs
 URT- nasal congestion, sinusitis, hoarseness
 Cardiac- arrythmia, DCMP, sudden death
 Neuro- cranial n. palsy-VII, VIII, IX, II, III, V, aseptic
meningitis, SOL, polyneuropathy,
mononeuritis multiplex
 HSmegaly, PLNE, arthralgias,
parotid/lacrimal gland enlargement/dysfunction


4. Symptom complex
 Lupus pernio- disfiguring facial cutaneous
involvement with violaceous plaques &
nodules
 Lofgren syndrome- acute presentation with
erythema nodosum, B/L hilar LNE, uveitis,
polyarthritis
 Heerfordt syndrome-acute presentation with
fever, parotod/lacrimal gland enlargement,
uveitis, B/L hilar LNE, cranial neuropathies

5. Diagnosis
Biopsy of most easily accessible
tissue
Showing non-caseating
granulomatous inflammation

6. Diagnosis- supportive
 CxR- helps in staging
 CT chest
 PET scan
 PFT
 RFT, LFT, Ca levels
 Serum ACE levels
 Negative Montoux test
 ECG
 MRI brain- for CNS involvement

7. Staging
 Based on CxR
 I- B/L hilar LNE
 II- + reticulonodular infiltrates
 III- B/L pulmonary infiltrates
 IV- fibrocystic disease
 Pleural effusion is rare
 DDx- TB, fungal infection, lymphoma,
Wegener’s granulomatosis

8. Treatment
 Not all need to be treated, ~50% need
 Spontaneous remission is known
 Indications-
 Persistent, symptomatic, progressive lung disease
 Disfiguring skin disease or peripheral LNE
 Threatened organ failure- eye, heart, CNS
 Persistent hypercalcemia, deranged RFT/LFT
 Uveitis, not responding to local steroids
 Hypersplenism

9. Drugs
Steroids- oral prednisone
x 1 year/more
Steroid-sparing agents, if required

10. Prognosis
 Variable
 Spontaneous remission is known
 Chronic disease, with exacerbations &
remissions
 Can progress to fibrosis, cor pulmonale,
respiratory failure
 Increased risk of lymphoma
 Systemic, specially cardiac involvement,
suggests bad prognosis