SESSION TITLE: Miscellaneous Student/Resident Case Report Posters I
SESSION TYPE: Medical Student/Resident Case Report
PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM
INTRODUCTION: We report a case of a 77 year old female who presented with hemoptysis and a purpuric rash found to have Henoch Schonlein Purpura.
CASE PRESENTATION: A 77 y/o female with no significant pulmonary disease presented with 3 episodes of hemoptysis within 48 hours, associated with onset of a purpuric rash on her legs 3 days prior to the hemoptysis, predominantly on the left side, which later spread to the abdomen. Her platelet count was 225, INR 1.0, and platelet function analysis was normal. CTA showed two 3 and 4 mm nodules and calcified granulomas without any evidence of pulmonary embolism or lung mass. Bronchoscopy showed edema in right and left lower lobes, petechial lesions in the right main-stem bronchus and a blood clot, confirmed by biopsy, in the right lower lobe. The clot was successfully removed using cryotherapy without any evidence of residual abnormal mucosa or bleeding beneath the clot. Bronchoalveolar lavage was done which progressively returned bloody fluid suggestive of a diffuse alveolar hemorrhage. Punch biopsy of the purpuric lesions on the left thigh revealed dense neutrophilic infiltrate in the epidermis and the superficial dermis, distributed in the interstitial pattern compatible with leukocytoclasis with scattered eosinophils. Immunoflorescence studies revealed fine granular deposition of IgA, C3,fibrin and lambda chains in small vessels of the papillary dermis in consistency with IgA vasculitis.
DISCUSSION: Even though HSP is the most commonly seen vasculitis in children, 10% of HSP cases are reported in adults where it usually presents with a purpuric rash, abdominal pain, arthritis and renal disease (initial findings of proteinuria or hematuria). Pulmonary involvement in HSP is rare. It occurs more often in adult onset HSP and usually presents with diffuse alveolar hemorrhage (DAH), usual interstitial pneumonia or interstitial fibrosis. The presence of DAH is associated with high mortality and morbidity in patients with HSP, with almost 32% of the reported cases dying from the DAH. Treatment of adults and HSP is usually with supportive care, with steroids and cyclophosphamide showing some benefit in further progression of disease in some studies.
CONCLUSIONS: This case stresses the importance of recognizing HSP as a potential cause of DAH. Adults with HSP are also at an increased risk for developing significant renal involvement including end-stage renal disease. A study showed that almost 40% of adults diagnosed with HSP will develop ESRD or severe renal insufficiency within 14.8 years of diagnosis. Timely diagnosis of HSP in adults, monitoring the renal function and management with steroids and cyclophosphamide could prevent a fatal episode of pulmonary hemorrhage or renal failure and could be life saving in these patients.
Reference #1: Pillebout E, Thervet E, Hill G, Alberti C, Vanhille P, Nochy D: Henoch-Schönlein Purpura in adults: outcome and prognostic factors. J Am Soc Nephrol. 2002;13(5):1271-8.
DISCLOSURE: The following authors have nothing to disclose: Wajihuddin Syed, Raju Chelluri, Gaurang Vaidya, Shalin Kothari, Leena Pawar, Housam Hegazy
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