A case report of severe visceral beta2-microglobulin-derived amyloidosis without obvious joint symptoms or radiological findings in a chronic hemodialyzed patient with systemic lupus erythematosus.

Beta-2-microglobulin-derived amyloidosis (Abeta2M amyloidosis) is a critical complication for patients undergoing long-term renal replacement therapy. Osteoarticular lesions, rather than visceral organs, are susceptible to this type of amyloidosis, and the visceral form seems to occur at a relatively late stage. Herein, we report a case of severe visceral Abeta2M amyloidosis without obvious joint symptoms or radiological findings in a chronic hemodialyzed patient who had received long-term treatment with glucocorticoid steroids for systemic lupus erythematosus.

Renal lesions in IgG4-related systemic disease.

Objective: Recently, a new concept of IgG4-related systemic disease including autoimmune pancreatitis, characterized by a high serum IgG4 level and tissue infiltration by IgG4-positive plasma cells, has been proposed. Our aim was to investigate the renal involvement in this condition.

Patients And Methods: We investigated the results of laboratory and imaging studies of the kidneys in 7 patients with IgG4-related systemic disease, and examined the renal histology in four of them.

Tubulointerstitial nephritis associated with IgG4-related systemic disease.

We report three patients with tubulointerstitial nephritis (TIN) with high serum IgG4 concentrations. None of the patients had notable pancreatic lesions when the TIN developed, although one had a history of autoimmune pancreatitis (AIP). Nevertheless, the clinicopathological findings were quite similar to those of AIP.

Lymphoplasmacytic infiltration of multiple organs with immunoreactivity for IgG4: IgG4-related systemic disease.

We report three elderly men with high serum IgG4 concentrations and multiple lymphoplasmacytic inflammation of the salivary glands, lymph nodes, pancreas, and renal interstitium. The infiltrating plasma cells had strong immunoreactivity for IgG4, even in patients without pancreatic lesions. These cases show that IgG4 is associated not only with autoimmune pancreatitis, but also with other systemic lymphoplasmacytic disease.

Syndrome of inappropriate secretion of antidiuretic hormone and thrombocytopenia caused by cytomegalovirus infection in a young immunocompetent woman.

We report the first case of the syndrome of inappropriate secretion of antidiuretic hormone (SIADH) associated with cytomegalovirus (CMV) infection. A 32-year-old woman was admitted to our hospital because of pandysautonomic signs and symptoms. Thrombocytopenia and hyponatremia were present.

Two cases of hypertrophic pachymeningitis associated with myeloperoxidase antineutrophil cytoplasmic autoantibody (MPO-ANCA)-positive pulmonary silicosis in tunnel workers.

Two cases of hypertrophic pachymeningitis (HP) associated with pulmonary silicosis in tunnel workers are described. In both cases the myeloperoxidase antineutrophil cytoplasmic autoantibody (MPO-ANCA) was positive. Two patients with pulmonary silicosis developed headache and neurological disturbance, and a diagnosis of HP was made.

Four cases of pheochromocytoma in patients with end-stage renal disease.

Although pheochromocytoma in a patient with end-stage renal disease (ESRD) is considered extremely rare, we recently encountered 4 ESRD patients with pheochromocytoma. Three were symptomatic, and in the fourth patient the tumor was discovered as an adrenal incidentaloma. Plasma catecholamine levels were significantly increased in two patients.