The association between IgA nephropathy(IgAN) and pulmonary capillaritis is rare. We present a unique case where the IgAN had progressed to end-stage renal failure before developing diffuse alveolar hemorrhage(DAH) which was treated with plasma exchange, systemic steroids and cyclophosphamide. This highlights the varied clinical course of IgAN and successful outcome with aggressive immunosuppression.
A 20-year-old Chinese male presented with small volume fresh hemoptysis and progressive dyspnea for two weeks. There were no other symptoms or contact history with tuberculosis. His drug history was unremarkable. There was no improvement despite a prior course of oral antibiotics. He was hemodialysis dependent from IgAN that was diagnosed by immunofluoresence on renal biopsy three years earlier.On examination he was febrile, tachycardic and tachpneic. There were no skin lesions. Bilateral coarse crepitations were heard on ascultation.Chest radiograph showed extensive bilateral alveolar infiltrates.(figure 1) Hemoglobin was 6.9 grams per deciliter(baseline 8.7). The white-cell count was 8110 per cubic millimeter with 85% neutrophils. Coagulation profile and electrolytes were normal. PaO2 was 84 mmHg on 40% supplemental oxygen.Community acquired pneumonia was the initial diagnosis with a differential of DAH. Intravenous ceftazidime, erythromycin and cloxacillin were initiated. Bronchoscopy on day two identified fresh blood throughout the tracheobronchial tree. Bronchoalveolar lavage(BAL) from the middle lobe obtained progressively bloodier returns. He was too dyspneic to undergo transbronchial lung biopsy.Blood, sputum and BAL work-up for sepsis was negative. Abundant neutrophils and hemosiderin-laden macrophages were noted on BAL cytology. Serum anti-nuclear, anti-basement membrane and anti-neutrophil cytoplasmic antibodies were negative. Serum complement level(CH50) was 41(normal 60-153).Daily intravenous methylprednisolone at one gram and plasma exchange of 2.5 liters was initiated for the next three days. The hemoptysis rapidly resolved and he was weaned off oxygen. He was discharged on oral prednisolone at one milligram(mg) per kilogram per day.After declining open lung biopsy, a transbronchial lung biopsy revealed acute inflammation that was consistent with pulmonary capillaritis.(figure 2) Cyclophosphamide(50 mg/day) was added after a second hospitalization for frank hemoptysis. He has been in remission for a year.
All previous reports indicate pulmonary involvement in IgAN either coincides with renal disease or precedes it. Our patient had already progressed to renal failure and the immune pathology in the kidneys was quiescent. Subsequent development of an immune mediated capillaritis suggests that this is an independent manifestation of the same underlying disease process.The prognosis of pulmonary capillaitis in IgAN is poor with only three survivors[1,3,5] among seven previously reported cases.[1-5] Plasma exchange and azathioprine therapy is reported in one case, but it was unsuccessful. All the other cases were managed with either steroids[1,345] or supportive care. Ours is the first report of cyclophosphamide as adjunctive therapy to steroids in DAH related to IgAN.
Although bleeding tendencies are common in uremic patients, the rare association between IgAN and pulmonary capillaritis must not be overlooked. Occurrence of DAH is independent of the stage of renal disease. Aggressive immunosuppression can result in a good outcome.
D. Anatham, None.