Scleroderma Renal Crisis Associated With Microangiopathic Hemolytic Anemia in a Patient With Seronegative Scleroderma and Monoclonal Gammopathy.

Systemic sclerosis with negative serology, particularly that complicated by scleroderma renal crisis (SRC), is rarely encountered. We describe a patient with seronegative systemic sclerosis who developed acute kidney injury, proteinuria, and hypertensive emergency following motor vehicle-related trauma and in the setting of nonsteroidal anti-inflammatory drug use. Findings on physical examination, imaging, and skin biopsy led to a clinical diagnosis of scleroderma, despite the lack of supportive laboratory data. IgG lambda paraproteinemia was detected on workup. Bone marrow biopsy showed plasmacytosis and trace lambda-restricted plasma cells consistent with monoclonal gammopathy of undetermined significance. Chemotherapy was initially started given concern for myeloma with cast nephropathy but was later stopped after a kidney biopsy revealed thrombotic microangiopathy (TMA). The SRC associated with TMA was ultimately diagnosed, though atypical hemolytic uremic syndrome (aHUS) induced perhaps by monoclonal gammopathy or hypertension was also possible. Captopril and eculizumab were initiated for SRC and aHUS, respectively. Despite therapy, renal function did not recover, and the patient required hemodialysis indefinitely. This case highlights clinical features common to both SRC and aHUS as well as points out a few ways to differentiate between them.

Characteristics and outcomes of HIV-1-infected patients with acute respiratory distress syndrome.

PURPOSE: We determined the prevalence of risk factors for the development of acute respiratory distress syndrome (ARDS), outcomes of critical illness, and the impact of highly active antiretroviral therapy in HIV-1-infected patients. We hypothesized that in an urban county hospital, HIV-1-infected patients with ARDS would have a higher mortality than their HIV-1-uninfected counterparts. MATERIALS AND METHODS: Subjects were enrolled between 2006 and 2012. Baseline patient demographics, comorbidities, illness severity, causes of ARDS, and clinical outcomes were obtained. The primary end point was hospital mortality. RESULTS: A total of 178 subjects with ARDS were enrolled in the study; 40 (22%) were infected with HIV-1. The median CD4 count was 75 (15.3-198.3), and 25% were on highly active antiretroviral therapy. HIV-1-infected subjects were significantly younger (44 vs 52 years; P < .01) and had higher rates of asthma, chronic obstructive pulmonary disease, pneumonia, history of hospital-acquired infections, and prior sepsis. HIV-1-infected subjects had greater illness severity by Acute Physiology and Chronic Health Evaluation II scores (29 [24-31] vs 24 [22-25]; P < .01). Hospital mortality was not higher among HIV-1-infected subjects compared with HIV-1-uninfected subjects (50.0% vs 38.4%; P = .19). CONCLUSIONS: In patients with ARDS, HIV-1 infection was associated with greater illness severity but was not associated with higher mortality in ARDS. Future studies need to be done to evaluate the factors that contribute to high morbidity and mortality in medically vulnerable populations who develop ARDS.