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.

Objective. Patients with rheumatoid arthritis (RA) have higher incidence of cardiovascular diseases (CVDs) compared with age-and sex-matched controls. The objective of our study was to measure the knowledge of patients with RA about the association between their disease and cardiovascular (CV) risk and to measure the frequency of counseling by physicians based on patient report.Methods. A telephone survey was conducted among patients with RA enrolled in the Consortium of Rheumatology Researchers of North America RA registry to collect data on medical and social history and on knowledge about CVD risk in RA and how they learned about that risk. Multivariable logistic regression models were performed to determine the factors associated with patients' knowledge and factors influencing likelihood of physician counseling. The odds ratios (ORs) represent adjusted multivariable results.Results. Of 185 patients with RA included in the study, 87 patients (47%) were aware that RA was a CV risk factor. Older age (OR 0.6; 95% confidence interval [CI] 0.4-0.8 per decade) and smoking (OR 0.4; 95% CI 0.1-0.9) were associated with low awareness, whereas disease duration of more than 10 years (OR 5.2; 95% CI 2.2-12.1) was positively associated with patient knowledge. Counseling by physicians, mostly rheumatologists, on CV risk in RA was reported by 47 patients (25%). Disease duration of more than 10 years (OR 3.9; 95% CI 1.2-13.1) was positively associated with patient-reported counseling. Patients with hypertension were less likely to report counseling (OR 0.4; 95% CI 0.2-0.9).Conclusion. Our study demonstrated low patient awareness of CV risk with RA and low rates of patient-reported counseling by physicians. This is an unmet need in clinical practice, which may be overcome by multimodal approaches such as developing websites, organizing symposiums, and involving health care providers at various levels.

We describe a patient who was admitted to our medical centre with acute renal failure, hyponatraemia and hyperkalaemia. CT of the abdomen and pelvis showed a retroperitoneal mass with bilateral ureteral obstruction. Biopsy revealed fibrosis with inflammatory infiltrate, but rare IgG4-positive plasma cells. After placement of bilateral pigtail nephrostomy catheters, renal failure improved but metabolic derangements remained. Morning serum cortisol level was equivocal, but with blunted response on cosyntropin stimulation testing indicating adrenal insufficiency. Serology for 21-hydroxylase antibodies was strongly positive, supporting the diagnosis of Addison’s disease. In addition to nephrostomy catheters for obstructive uropathy, idiopathic retroperitoneal fibrosis was treated with mycophenolate mofetil. Physiological doses of hydrocortisone and fludrocortisone for Addison’s disease were also initiated. The patient continues to be monitored for regression of the mass. Based on review of the literature, this is the first reported case of IgG4-negative idiopathic retroperitoneal fibrosis presenting with autoimmune primary adrenal insufficiency.

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