Calciphylaxis: a case series and the role of radiology in diagnosis.

BACKGROUND: Calciphylaxis is a syndrome of vascular calcification most commonly affecting patients with end-stage renal disease (ESRD) on hemodialysis. Because of its high mortality rate, early diagnosis and treatment are necessary. Although diagnosis is usually based on skin biopsy, histopathology is often nonspecific. As the role of imaging in calciphylaxis has not been studied extensively, we examined the utility of radiology in the diagnosis of this disease. METHODS: A thorough review of electronic medical records for 2005-2013 at Loyola University Medical Center yielded 10 patients with biopsy-proven calciphylaxis. Using the radiological picture archiving and communication system (PACS), all imaging studies of the affected body part obtained within 6 months of diagnosis were analyzed and tabulated. RESULTS: All 10 patients had undergone imaging (computed tomography, ultrasound, plain radiography, and/or mammography) of the affected anatomy prior to diagnosis by skin biopsy. Nine of these patients were noted to have moderate-to-severe soft tissue vascular calcification in the area of skin biopsy. CONCLUSIONS: This case series supports the suggestion that findings of superficial vascular calcifications on imaging studies are sensitive for the diagnosis of calciphylaxis. Used in conjunction with histopathological, clinical, and laboratory data, radiology can serve an important role in the diagnosis of calciphylaxis.

Calciphylaxis: a systematic review of existing and emerging therapies.

Calciphylaxis, also known as calcific uremic arteriolopathy, is a cutaneous ischemic small vessel vasculopathy seen in 1 to 4% of patients with chronic kidney disease on hemodialysis. It is associated with extreme pain and a 60 to 80% mortality rate in the setting of few and frequently ineffective therapeutic options, although this may be changing based on reports of success with newer therapies.

Infliximab.

Infliximab is a chimeric monoclonal antibody that interferes with the actions of the pro-inflammatory cytokine tumor necrosis factor-alpha (TNF-alpha). Clinical trials of infliximab have demonstrated a rapid and substantial response in patents with psoriasis and psoriatic arthritis, substantiating the role of TNF-alpha in the immunopathogenesis of psoriatic disease. This review summarizes the current data regarding the use of infliximab in treating psoriasis and psoriatic arthritis, as well as the safety data available from patients with other immune-mediated inflammatory disorders. Practical issues such as patient selection, monitoring, cost, and potential combination therapies are also discussed.