Quiz Page April 2009: Kidney Failure, Gross Hematuria, and Hemoptysis in a Diabetic Man Undergoing Treatment for Non-Hodgkin Lymphoma

What is the differential diagnosis for this presentation? 

Our patient presented with a triad of hemoptysis, hematuria, and acute kidney injury. Given his other autoimmune diseases, pulmonary-renal syndrome caused by autoimmune vasculitis was suspected. The term pulmonary-renal syndrome refers to the combination of diffuse alveolar hemorrhage and rapidly progressive glomerulonephritis. Patients may present with severe respiratory and/or kidney failure. A variety of mechanisms, such as those involving anti–glomerular basement membrane antibodies, antineutrophil cytoplasm antibodies, immunocomplexes, and thrombotic microangiopathy, are implicated in the pathogenesis of this syndrome. Because the syndrome is characterized by a fulminant course if left untreated, early diagnosis and timely initiation of treatment are crucial for the patient's outcome. In our patient, pulse steroid therapy was started before confirming an autoimmune process.

What is the appropriate diagnostic and therapeutic approach in this case, and are there special considerations in immunocompromised patients? 

In a patient with clinical suspicion for pulmonary-renal syndrome, the presence of glomerulonephritis and pulmonary hemorrhage should be confirmed, ideally with bronchoscopy and evidence of RBC casts in the urine sediment, while awaiting results of a kidney biopsy. In our patient, chest x-ray and computed tomographic scan showed a cavitary consolidation of the left upper lung lobe (Fig 1), and urine sediment showed numerous RBCs without casts. These findings are not consistent with the diagnosis of pulmonary-renal syndrome and suggested other causes of lung and kidney disease. Results of additional workup, including antinuclear antibody, antineutrophil cytoplasmic antibody, prothrombin time, partial thromboplastin time, hepatitis panel, human immunodeficiency virus antibodies, anti–glomerular basement membrane antibodies, ASO, complement titers, erythrocyte sedimentation rate, C-reactive protein, sputum smears for acid-fast bacilli (AFB), purified protein derivative (tuberculin) testing, and assays Mycobacterium tuberculosis infection (QuantiFERON; Cellestis Limited, Carnegie, Australia), Aspergillus antibodies, and Cryptococcus antigen were all within normal limits. The kidney biopsy showed invasive fungal infection consistent with zygomycosis, infarction secondary to arterial fungal thrombosis, and ATN (Fig 2). Steroid therapy was stopped, and the patient was started on liposomal amphotericin B. Fungal culture results grew Rhizopus species. The patient required dialysis and died shortly afterward.

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Figure 1. Computed tomographic scan of the chest.

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Figure 2. Kidney biopsy specimen (hematoxylin and eosin stain; original magnification ×200).

Opportunistic infections should be considered in immunocompromised patients presenting with hemoptysis, hematuria, and acute kidney injury.