DIAGNOSIS IN DIFFUSE ALVEOLAR HEMORRHAGES

1. DIAGNOSIS IN DIFFUSE ALVEOLAR HEMORRHAGES Demet KARNAK, MD, Professor Ankara University School of Medicine Chest Disease Department

2. DIFFUSE ALVEOLAR HEMORRHAGES • Hemorrhage to alveolar spaces • Alveolo-capillary membrane damage • Damage to arterioles-venules and alveolar septal lines along with tears in capillaries around these areas • Even if excess hemorrhage exist, hemoptysis can be absent?

3. ETIOLOGY and HISTOLOGY • Pulmonary capillaritis • Bland Pulmonary Hemorrhage  • Diffuse Alveolar Damage

4. Pulmonary capillaritis • Interstitial neutrophilic infitration reflecting alveolar septas • Rapid necrosis, advanced structural discrepancies and red blood cell invasion in the alveolar spaces • Neutrophils fragment in a short while • Toxic oxygen radicals can be the cause ? • Fragmented neutrophils, nuclear dust and fibrine migrate to the alveolar space leading to fibrinoid necrosis

5. Pulmonary capillaritis

6. Capillaritis

7. Bland Pulmonary Hemorrhage • Alveolar hemorrhage without inflammation and destruction

8. Bland Pulmonary Hemorrhage

9. Diffuse Alveolar Damage • Underliying form of acute respiratory distress syndrome • Characterized by alveolar septal edema and hyaline membrane formation • This scenario is also DAH

10. Diffuse Alveolar Damage

11. Diffuse Alveolar Damage

12. Connective Tissue Disease

13. Other Diseases

14. Other Diseases

15. CLINICAL PRESENTATION • Dyspnea • Hemoptysis • Increased alveolo-arterial oxygen gradient • Anemia • Diffuse alveolar pattern-in chest x-ray • DLCO (first 48 h) • BAL findings

16. DAH-Chest x-ray

17. Bronchoscopy • Hemorrhagic feature of the BAL fluid • hemosiderin pigment in alveolar macrophages (prussian blue) • Exclude DAH if BAL findings are absent • TBB can be helpful in differential diagnosis especially in granulomatous disease or vasculitis

19. CLINICAL VIEW

20. HISTORY • Drug addiction • Connective Tissue disease • İmmune status • Vasculitic symptoms • Cardiac pathologies

21. SYMPTOMS and SIGNS • Usually no more than a week • Cough, dypnea, fever are the most common symptoms • Meed of mechanic ventilation in some cases • Hemoptysis can be absent in 33% on the first presentation • Localized or diffuse alveolar infiltration, Hb drop and hemorrhagic BAL fluid can suggestive for DAH

22. PHYSICAL EXAMINATION • Usually nonspecific • Difficult to diagnose if there is no underlying disease such as vasculitis or connective tissue disease! • Uveitis or leukocytoclastic vasculitis collagen vascular disease, mitral stenosis can be easily caught!

23. RADIOLOGY • Chest X-ray can be non-spesific • Diffuse patchy alveolar opacities • Recurrent DAH episodes- lung fibrosis and persistent interstitiel infiltrates • COPD+DAH • MPA+DAH

24. LABORATORY ANALYSES • ESR • Leukocytes • HTC • Pulmonary-renal disease • Glomerulonephritis • Elevated plasma creatinin concentration • Urinalysis: Proteinuria, leukocytes and erithrocytes

25. DAH TRIAD FOR DIAGNOSIS • Gas exchange abnormalities-hypoxemia • Need of mechanical ventilation ?! • Increased DLCO • Dıffuse alveolar infiltration

26. SPECIAL OCCASIONS • ANCA antibodies can be helpful in the diagnosis of Wegener Granulomatosis, MPA, idiopathic pulmonary-renal syndrome • Cytoplasmic ANCA (c-ANCA), reflects antiproteinase 3 (PR 3) and mostly specific for Wegener Granulomatosis • Perinuclear ANCA (p-ANCA) reflects anti-myeloperoksidase antibody and meaningful in the diagnosis of MPA and idiopathic pulmonary-renal syndrome

27. SPECIAL OCCASIONS • Untreated SLE cases has 95% ANA antibody positivity • Anti-ds DNA, is positive in the 50-75% of the cases • Anti-ds DNA positivity and decreased C3 level is 100% diagnostic for SLE • Tissue immunoflorescent studies and serum ABMA (antiglomerular basal membrane antibody) measurement is 97% sensitive and 98% specific for Goodpasture syndrome

28. TREATMENT • Different according to underlying etiology

29. PROGNOSIS • Recurrent episodes cause to irreversible pulmonary fibrosis • Idiopathic pulmonary hemosiderosis, wegener, and mitral stenosis can be examples of this scenario • After DAH, severe obstructive pulmonary damage and emphysema were reported

30. MORTALITY • Related to underlying cause • SLE, anti-GBM disease and Idiopathic pulmonary hemosiderosis has 25-50% early mortality rate

31. Mortality

32. 35 year old female High fever, night sweeting, trembling and fatigue for ten days Severe dypnea for 2 days Loss of weight 7kg/month Hypertyroidism 6 year ago, taking PTU pills Beta-blocking agent Anti-depressant DAH-PTU

34. 1. Ay kontrol 6. Ay kontrol

35. 6. Ay kontrol BT

36. DAH-CMV pneumonia

37. DAH-MPA

38. SAH-MPA

39. DAH-CMP