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- 1. Dr. Manu Mohan. K Pulmonary Medicine
- 2. Systemic disease of unknown cause Multisystem involvement
Granulomatous inflammation of the lung
- 3. Epidemiology Commonest in North American blacks North
European whites Women > male. Onset 3rd or 4th decade.
- 4. Etiology & pathogenesis Unknown antigen activates
alveolar macrophages and helper T cells Release of monokines
Attract monocytes and proliferation of B cell B cell activated to
form plasma cells. Granulomas soft noncaseating
- 5. Clinical Features Symptoms One third asymptomatic
Respiratory cough, dyspnoea Cutaneous and ocular Fatigue, fever,
night sweats and weight loss
- 6. Lofgren syndrome - Erythema nodosum, arthralgias and
bilateral hilar adenopathy. Heerfordt syndrome fever, parotid
enlargement, anterior uveitis and facial nerve palsy.
- 7. Clinical Features Chest crackles uncommon. Erythema nodosum
Parotid gland enlargement Hepatosplenomegaly Lymphadenopathy
- 8. Lab Investigations Leukopenia, ESR increased. Hypercalcemia
(5%) Hypercalciuria (20%) ACE level elevated (40-80%)
- 9. Pulmonary Function Test Evidence of air flow obstruction
Restrictive changes with decreased lung volumes and diffusing
capacity are common.
- 10. Mantoux Test Skin test anergy in 70%. Tuberculin test
depressed or absent.
- 11. Radiography Stage I bilateral hilar adenopathy alone Stage
II hilar adenopathy and parenchymal involvement. Stage III
parenchymal involvement alone Stage IV - fibrosis
- 12. Chest X-ray Parenchymal shadows are diffuse reticular
infiltrates focal infiltrates acinar shadows, nodules cavitation
Pleural effusion