SESSION TITLE: Critical Care Case Report Posters II
SESSION TYPE: Affiliate Case Report Poster
PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM
INTRODUCTION: Scleroderma is an autoimmune disease than can involve multiple organs. Life threatening complication includes pulmonary renal syndrome (acute renal failure and diffuse alveolar hemorrhage “DAH”) and scleroderma renal crisis “SRC” (acute renal failure and malignant hypertension).
CASE PRESENTATION: 50 year old Caucasian female with past medical history of Scleroderma presented with confusion, headache, and bilateral vision loss and gait disturbances. Physical exam was positive for severe hypertension with BP of 220/190 and confusion. Rest of the exam was unremarkable. Laboratory studies were: WBC of 16000 , HGB11 g/dl , PLT 58 k/uL, INR 2.4, ESR of 45, BUN of 52 mg/dl, creatinine of 3.7 mg/dl, LDH of 1781 IU/L and Haptoglobin of < 30 mg/dl and peripheral blood smear with 3-5 schistocytes suggestive of Microangiopathic hemolytic anemia. CXR showed bilateral airspace disease. CT scan of the head revealed bilateral hypodensities in the occipital lobes and cerebellum suggestive of posterior reversible encephalopathy syndrome. She was admitted to the ICU and started on a labetalol drip to control BP and hemodialysis secondary to anuric renal failure. The patient’s condition declined despite adequate BP control and she required intubation and mechanical ventilation. Bronchoscopy with bronchoalveolar lavage was consistent with DAH. Plasmapheresis was initiated empirically for pulmonary renal syndrome and TTP while the work up was in progress. IV steroids were not started to avoid further deterioration in her condition in case this was SRC with DAH. Further investigation revealed a positive ANA with nucleolar pattern, low complement 4 level, negative ANCA, high renin level of 14 ng/ml/hr and a normal ADAMSTS 13 level. Renal biopsy confirmed thrombotic microangiopathy and no evidence of vasculitis. Patient was started on an angiotensin converting enzyme inhibitor (ACEI). Her mental and respiratory status improved and she was extubated and transferred out of the ICU.
DISCUSSION: DAH and Pulmonary renal syndrome in the setting of scleroderma raises the suspicion of vasulitis. However, DAH has been described in SRC. While corticosteroids are beneficial in the setting of DAH secondary to vasculitis, they are harmful in the setting SRC. ACEI and supportive care is the main treatment for SRC.
CONCLUSIONS: Not all cases of DAH and pulmonary renal syndrome in scleroderma are caused by vasculitis. The diagnosis of vasculitis and exclusion of SRC should be confirmed before initiating corticosteroid therapy.
Reference #1: Cossio M et al. Life-threatining complication of systemic sclerosis. Crit Care clin. 2002; 18: 819-839.
DISCLOSURE: The following authors have nothing to disclose: Brendon Colaco, Khaled Khasawneh
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