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