Lethal necrotizing granulomatous vasculitis after bariatric surgery and bilateral mammaplasty

The aim of case presentation. To present a typical case of systemic autoimmune disease related to extremely aggressive medical alterations of the immune status which were carried out not only for life-saving indications, but for cosmetic reasons as well. Summary. The patient (32-year-old female) had a history of persistent irregular febrile fever for two years, renal disease (tubulointerstitial nephritis with papillary necrosis), hemorrhagic vasculitis-like skin involvement and mediastinal lymphadenopathy. Patient underwent multiple investigations aimed for ruling out infectious causes of the disease that yielded negative result, repetitive courses of antibiotics provided transient positive effect at onset of disease. Five years before the origin of symptoms patients underwent sleeve gastrectomy with biliopancreatic shunting for obesity (body weight - 130 kg, BMI - 43.4 kg/m2), cholecystectomy and appendectomy. After that within a year patient lost 55 kg of body weight and developed severe symptoms of malabsorption. In 1.5 years after the fever onset bilateral mammaplasty with silicone implants was carried following which the fever became constant. At transthoracic biopsy of mediastinal lymph nodes demonstrated the pattern of Wegener-type ANCA-negative necrotizing granulomatous vasculitis of small arteries (PCR for mycobacteria and acid fast stain were negative). Despite provided corticosteroid and cyclophosphan treatment, the disease progressed (focal affection of the lungs) and the patient died of brain stem hemorrhage. Autopsy revealed the signs of vasculitis with involvement of the brain, skin, mediastinal lymphnodes, lungs, as well as the signs of chronic inflammation, atrophy of breast gland lobules, embolization of the focal lymphatics by the implant substance, ulceronecrotic enteritis of defunctioning bowel, severe liver steatosis with the chronic vitamin deficiency. Conclusion. The relation of atypical necrotizing vasculitis with yet undescribed clinical presentation with previous bariatric surgery and long-standing malabsorption is highly probable; application of silicone implants with adjuvant-associated immune response were additional resolving factors. Incomplete effect of immunosuppressive therapy indicates the dominant role of autoinflammation in the course of disease. Selection of patients for bariatric and cosmetic surgery should be more careful and involve estimation of the general and immune status of the body.

гранулематозный васкулит, продольная резекция желудка, билиопанкреатическое шунтирование

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