Pulmonary Manifestations of Systemic Disease: Fellow Case Report Poster - Pulmonary Manifestations of Lung Disease |

Diffuse Alveolar Hemorrhage in a Patient With Atypical Hemolytic Uremia Syndrome FREE TO VIEW

Faisal Zahiruddin, DO; Janice Zimmerman, MD
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Houston Methodist Hospital, Houston, TX

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):1059A. doi:10.1016/j.chest.2016.08.1166
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SESSION TITLE: Fellow Case Report Poster - Pulmonary Manifestations of Lung Disease

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Atypical hemolytic uremic syndrome (aHUS) is a rare disease that is associated with microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury (AKI). It is rarely accompanied by diffuse alveolar hemorrhage (DAH) and only a few cases have been reported.

CASE PRESENTATION: A 20 year-old female with no significant past medical history presented with 3 days of cough and shortness of breath. She also endorsed 4 weeks of fatigue, decreased appetite, chest pain, and subjective fevers. She denied chills, night sweats, weight loss, travel, or pets. She was diagnosed with a viral syndrome on her first emergency department visit and discharged without testing. She returned 2 days later with the same complaints. Vital signs were BP 151/94, HR 110, RR 15, T 99, SpO2 85%. Significant physical examination findings were rhonchi with decreased breath sounds at the bases. She was intubated for respiratory failure and blood-tinged secretions were noted in the endotracheal tube. Laboratory studies showed creatinine 26.6 mg/dL, BUN 145 mg/dL, bicarbonate 13 mEq/L, hemoglobin 9.4 g/dL, and platelet count 103 k/uL. Chest radiography revealed multifocal airspace infiltrates. On bronchoscopy, serial BAL aliquots were gradually more hemorrhagic. ADAMTS13 activity was 44%. Renal biopsy revealed thrombotic microangiopathy. Genetic studies showed heterozygous abnormality of complement factor H (CFH). She was initiated on dialysis, high dose steroids, plasma exchange, and eculizumab. She was extubated on day 6 and discharged home with outpatient dialysis and weekly eculizumab injections.

DISCUSSION: This patient had microangiopathic hemolytic anemia, thrombocytopenia, and renal failure, which are the hallmarks of aHUS. In addition, the aHUS was associated with the rare finding of DAH. CFH is associated with a poor prognosis in aHUS. However eculizumab, a monoclonal antibody that inhibits C5, can be used in cases that are refractory to plasma exchange. Multimodality treatment in this patient improved the hemolytic anemia, pulmonary infiltrates, and renal function.

CONCLUSIONS: Atypical HUS should be considered in the differential for patients presenting with DAH and AKI. Steroids, plasma exchange, and eculizumab can be used in the treatment.

Reference #1: Rhee H. Pandemic H1N1 Influenza A Viral Infection Complicated by aHUS And DAH. Clin Exp Nephrol 2011; 5:948-952.

Reference #2: Abdallah E. ANCA Negative Pulmonary Renal Syndrome with Pathologic Findings of Thrombotic Microangiopathy. Arab J of Nephrol and Transplantation 2013; 6(1):31-35.

DISCLOSURE: The following authors have nothing to disclose: Faisal Zahiruddin, Janice Zimmerman

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