A 37-year-old black male patient, previously healthy, came to our outpatient clinic with a nine-year history of persistent night fever and night sweating, associated with pain in the right hypochondrium radiating to the epigastrium. The patient also presented iron deficiency anemia refractory to iron replacement, high platelet counts and positive inflammatory markers (CRP and ESR).
Initially all infectious, autoimmune and neoplastic known causes were excluded. An abdominal CT revealed hepatosplenomegaly and retroperitoneal mass. High-dose corticosteroids prescribed empirically at high doses were only partially effective.Histopathological analysis of retroperitoneal mass was compatible with IgG4-RD. Steroid-sparing agents were not effective in controlling symptoms, as well as tapering steroids dosage was also not able to reduce the levels of inflammatory markers. For this reason, B cell depletion therapy was initiated (ritutximab 1g/dose every 4 weeks in two doses). Clinical control could be observed but without considerable reduction in inflammatory markers.
Due to the socioeconomic context, the patient had low adherence to the proposed treatments and to following the check-up routine.
Six years after diagnosis, he was admitted to the emergency room with severe right hypochondrium pain, after presenting hematochezia for twenty days.
Laboratory findings included a significant increase in inflammatory markers, hypogammaglobulinemia, hypoalbuminemia, high pro-BNP levels and nephrotic proteinuria with normal renal function. Imaging exams showed increased hepatosplenomegaly (Fig. 1). Also, hemorrhagic gastroduodenitis was diagnosed during an upper gastrointestinal endoscopy. Multiple specimen biopsies were obtained from esophagus, stomach, duodenum and liver. Amorphous material deposits were identified in all biopsy tissue samples, with a positive Congo red stain (Fig. 1). Subsequently, it was observed a serum amyloid A protein of 186 mg/L (reference value below 6.4 mg/L). Empirical treatment with colchicine was initiated and anti-IL6 was proposed. However, after two months of hospital discharge, and before anti-IL 6 could be started, he developed septic shock refractory to clinical measures progressing to death. All relevant laboratory data and treatment used are shown in Fig. 1.