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. It should be noted that prominent visceral Abeta2M amyloidosis can develop without any osteoarticular symptoms or radiological abnormalities in dialyzed patients undergoing prolonged glucocorticoid therapy.

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. All patients showed elevated serum IgG4 levels, and 4 had autoimmune pancreatitis. The other three patients showed involvement of various extrapancreatic organs (lymphadenopathy, sialadenitis or renal insufficiency), and abundant IgG4-positive plasma cell infiltration was confirmed in their affected tissues. RESULTS: Six of the 7 patients showed some renal abnormalities. In one patient, hydronephrosis was observed accompanied by retroperitoneal fibrosis. Another patient showed multiple low-density areas in both kidneys by computed tomography, and gallium citrate scintigraphy showed gallium-67 accumulation in both kidneys, although renal function was normal. Four patients had tubulointerstitial nephritis. In two of them, the tubulointerstitial nephritis was diffuse. In one patient, marked diffuse but patchily distributed lymphoplasmacytic infiltration of the renal interstitium was observed. In another patient, computed tomography showed a tumor-like low-density mass; open biopsy of the mass showed aggregates of lymphocytes and plasma cells in the renal interstitium. CONCLUSION: Renal parenchymal lesions in IgG4-related systemic disease are due to dense lymphoplasmacytic infiltration of the renal interstitium, and the lesions vary from diffuse tubulointerstitial nephritis to tumor-like masses according to the distribution patterns of the infiltrating cells.

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. They were all middle-aged to elderly men. Sialadenitis and lymphadenopathy were often evident. Serum total IgG and IgG4 concentrations were elevated and hypocomplementemia was observed. Although antinuclear antibodies were positive, anti-Ro and anti-La antibodies were negative. Renal biopsy showed dense lymphoplasmacytic infiltration with fibrosis in the renal interstitium, and the infiltrated plasma cells had strong immunoreactivity for IgG4. Furthermore, lymphoplasmacytic infiltration and abundant IgG4-positive plasma cells were observed in the salivary glands of a patient. Steroid therapy was effective for TIN in all three patients. The present findings support the recently proposed concept of IgG4-related systemic disease, and suggest that IgG4 is associated not only with AIP but also with other systemic lymphoplasmacytic diseases, including TIN. The conditions responsible for the pathogenesis of TIN need to be considered, irrespective of the presence 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.

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. In both cases the serum CRP level and the MPO-ANCA titer were elevated. Corticosteroid therapy produced a rapid improvement in all the clinical and laboratory parameters. Although an association has been noted between exposure to silica dust and ANCA-associated vasculitis, particularly glomerulonephritis, central nervous system involvement is rare. However, there have been some recent reports of HP cases that were positive for ANCA, and the association between HP and vasculitis has been discussed in the medical literature. HP may be one feature of multiorgan involvement in ANCA-associated disease, and the association between silica dust exposure and HP should be considered, as with other forms of ANCA-associated vasculitis.

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. Serum anti-CMV IgM and IgG antibodies were positive. Despite hyponatremia, urinary osmolality exceeded plasma osmolality and plasma vasopressin levels related to plasma osmolality were high. Restriction of water intake and administration of dimethylchlorotetracycline improved hyponatremia, suggesting this patient had SIADH. In this patient, SIADH may have been caused by acute pandysautonomia that developed following CMV infection.

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. In each case, 131I-MIBG scintigraphy showed accumulation of the radionuclide in the adrenal tumor, which was identified by MRI or CT scanning, and adrenalectomy was conducted without serious complications. Although paroxysmal hypertension is a common symptom in patients with ESRD, pheochromocytoma must be eliminated by careful evaluation.