Autoimmune diseases (AID) have been associated with a variety of lymphoproliferative disorders. Multiple myeloma (MM), one of the most common haematologic malignancies characterized by clonal proliferation of bone marrow plasma cells, has been associated with a range of autoimmune disorders. In this report, we described a case study of a patient admitted to our Internal Medicine Department for a bone marrow biopsy and myelogram due to a monoclonal peak observed by his general practitioner. However, at admission he presented typical giant cell arteritis (GCA) complaints, suggesting the coexistence of both diseases. The possible pathogenesis, as found in the literature, explaining the association will be discussed.
Sarcoidosis is a multi-systemic disease characterized by non-caseating granulomas in various organs. The aetiology is still unknown. Although the liver is the third most commonly affected organ, hepatosplenic sarcoidosis without lung involvement is very uncommon. There is a high frequency of certain autoimmune illnesses observed in sarcoidosis, but association with type 1 diabetes is infrequent. We present the case of a 31-year-old woman, with type 1 diabetes mellitus diagnosed 22 years before with a glycated haemoglobin (HbA1c) above 14%, diabetic nephropathy, retinopathy and neuropathy, hypercholesterolemia and beta thalassemia. She was medicated with an angiotensin-converting enzyme inhibitor, a dihydropyridine calcium antagonist and insulin. The patient presented with a 4-month history of tiredness, abdominal pain, weight lost and hepatosplenomegaly. Abdominal ultrasound revealed hepatomegaly with regular contours, diffuse heterogeneous texture, containing numerous nodules with slight enlargement of the spleen. Serum angiotensin converting enzyme (ACE) was 67 IU/L and a sedimentation rate of 120 mm/h. Computer tomography (CT) scan confirmed hepatosplenomegaly and suggested infiltration in both organs. Liver biopsies were compatible with sarcoidosis. After ruling other organ involvement, a diagnosis of isolated hepatosplenic sarcoidosis was provided and prednisolone (40mg/day) was started. After a few months the patient developed a corticoid-induced myopathy confirmed with electromyography. Prednisolone was reduced to 20mg/day and azathioprine (50mg/day) treatment initiated. After a 7-month treatment, chest-abdomen-pelvis CT scan showed a marked reduction of the nodularity and hepatosplenomegaly and after 1 year the patient was completely asymptomatic (HbA1c, 7.5%; ACE, 24IU/L). At 18-month follow-up there was no evidence of recurrence (HbA1c, 7%), with optimum glycaemic control with total daily insulin dose lowered to half. This is an uncommon case in which the treatment of hepatosplenic sarcoidosis with regression of sarcoid tissue can help explain the improvement of glycaemic control in this patient.
Figure 1. Abdominal-pelvic contrast-enhanced CT scan showing portal venous gas (A), pneumatosis intestinalis (B), gastroparesis (C) and superior mesenteric artery clot (D)
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