Diffuse Alveolar Hemorrhage



Fig. 34.1
Chest X-ray at initial presentation showing multifocal bilateral alveolar opacities



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Fig. 34.2
(a, b) Chest CT images performed after hemodialysis showing diffuse bilateral alveolar opacities


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Fig. 34.3
Sequential bronchoalveolar lavages starting left to right showing progressively bloody return



Question

What is the diagnosis?


Answer

Capillaritis with diffuse alveolar hemorrhage

This patient presented with hemoptysis confirmed to be diffuse alveolar hemorrhage and chronic renal failure. The initial suspected etiology was volume overload due to missed dialysis sessions due to the elevated BNP level. The patient was treated conservatively with dialysis and while his reported hemoptysis improved, his chest x-ray showed persistent alveolar infiltrates. The serologic analysis that confirmed the diagnosis included Antinuclear Antibody of 1:360, P-ANCA positivity, and antibody to Myeloperoxidase of greater than 8.0 units. The constellation of diffuse alveolar hemorrhage, renal involvement, and p-ANCA/MPO positivity confirmed a diagnosis of Microscopic Polyangiitis. Imaging of the sinuses showed no signs of inflammation of the upper respiratory tract. The patient was treated with prednisone and cyclophosphamide pulse dosing for six doses. He had one subsequent episode of hemoptysis requiring hospitalization 1 month after initial presentation. In general, he responded well to treatment and was weaned from corticosteroids over the next 3 months after completion of cyclophosphamide with improvement on chest imaging (Fig. 34.4).

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Fig. 34.4
(a, b) Chest CT images performed after 1 year of immunosuppressive treatment showing resolution of alveolar opacities



Principles of Management



Differential Diagnosis


The differential diagnosis for diffuse alveolar hemorrhage can be quite extensive ranging from autoimmune to coagulopathy to medications. Three characteristic patterns have been identified: Capillaritis, ‘Bland’ pulmonary hemorrhage, and alveolar bleeding due to another process [1, 2]. Capillaritis is the most common cause of DAH and is typically a result of antibody-mediated cell damage. Autoimmune conditions associated with DAH include antineutrophil cytoplasmic antibody (ANCA) associated vasculitides, Goodpasture Syndrome, Systemic Lupus Erythematosus (Table 34.1). Initial evaluation of a patient with hemoptysis and suspected diffuse alveolar hemorrhage typically includes fiberoptic bronchoscopy to identify a source of bleeding, identification of potential infectious etiologies, and serologic workup for autoimmune conditions.


Table 34.1
Causes of diffuse alveolar hemorrhage







































































Capillaritis

Granulomatosis with polyangiitis (GPA)

Churg-Strauss syndrome

Microscopic polyangiitis (MPA)

Isolated pauci-immune pulmonary capillaritis

Idiopathic pauci-immune glomerulonephritis

Primary immune complex-mediated vasculitis

Goodpasture’s syndrome

Henoch-Schonlein purpura

Systemic lupus erythematosus

Rheumatoid arthritis

Antiphospholipid antibody syndrome

Mixed connective tissue disease

Polymyositis/dermatomyositis

Essential cryoglobulinemia

Behcet’s disease

Acute lung transplantation rejection

Autologous bone marrow transplantation

Bland Pulmonary Hemorrhage

Idiopathic pulmonary hemosiderosis

Coagulopathy: anticoagulants, anti-platelet, thrombolytics, DIC

Mitral stenosis, pulmonary veno-occlusive disease

Infection: human immunodeficiency virus infection, infective endocarditis

Toxin or inhalation injury: isocyanates, crack cocaine, retinoic acid

Drug-associated disease: propylthiouracil, diphenylhydantoin, amiodarone, mitomycin,

D-penicillamine, sirolimus, methotrexate, haloperidol, nitrofurantoin, gold,

all-trans-retinoic acid (ATRA), bleomycin, montelukast, zafirlukast, infliximab

Alveolar bleeding due to another condition

Diffuse alveolar damage

Pulmonary embolism

Sarcoidosis

High-altitude pulmonary edema, barotrauma

Infection: invasive aspergillosis, cytomegalovirus infection, legionellosis, herpes simplex virus infection,

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Jul 20, 2017 | Posted by in Uncategorized | Comments Off on Diffuse Alveolar Hemorrhage

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