Association between scleroderma, renal cell carcinoma and membranous nephropathy.

We report a patient with scleroderma, renal cell carcinoma (RCC) and membranous nephropathy (MN). Certain clinical and laboratory features suggested that RCC caused or enhanced the other two conditions. A 55-year-old man developed scleroderma which progressed rapidly during its first 2 years with development of hypertension and acute renal failure, peak serum creatinine (SCr) 327 micromol/l (3.7 mg/dl) and partial improvement of the renal function (SCr 239 micromol/l or 2.7 mg/dl) after initiation of an angiotensin converting enzyme inhibitor. He subsequently developed nephrotic syndrome (urine protein excretion 9 gm/24-h) and progressive renal failure, with SCr 469 +/- 18 micromol/l (5.3 +/- 0.2 mg/dl). An anti-nuclear mitotic apparatus protein (NUMA) antibody, which is uncommon in scleroderma but has been linked to certain malignancies, was found in his serum. A left upper pole RCC was removed by heminephrectomy. MN was found in the renal parenchyma adjacent to the excised tumor. In the 3.5 years following surgery, the clinical manifestations of scleroderma have been arrested while the medications prescribed for this condition have been greatly reduced. Proteinuria is consistently less than 1 gm/24-h and 42 months after surgery serum creatinine was 256 micromol/l (2.9 mg/dl). Nutrition has also improved. Although this case may represent chance occurrence of three uncommon diseases (scleroderma, RCC, MN) in the same individual, the sustained improvement of the manifestations of scleroderma and MN after resection of the RCC contrasted to the rapid course of these conditions until the surgery, and the presence in the patient's serum of an autoantibody which is uncommon in patients with scleroderma, but has been linked to malignancy, suggest a pathogenetic relationship between the three conditions.

Severe proximal myopathy in advanced renal failure. Diagnosis and management.

Myopathies encountered in uremic patients may have different pathogenetic mechanisms and treatment. Secondary hyperparathyroidism may cause uremic myopathy responding to specific treatment. This study aimed at presenting a case illustrative of the clinical features, diagnosis and management of uremic parathyroid myopathy. A 66-year old man with renal failure from membranous nephropathy developed sensory signs of uremic neuropathy and progressive painless weakness of the pelvic girdle muscles bilaterally. Motor nerve conduction velocity was normal, electromyogram was consistent with a myopathic pattern, while muscle biopsy showed a pattern of atrophy more consistent with a neuropathic pattern. Serological tests for collagen vascular diseases and hyperthyroidism were negative, while serum muscle enzymes were not elevated and serum phosphate levels were not low. Serum parathyroid hormone level was grossly elevated, while serum calcium was mildly elevated in a small fraction of the measurements, serum alkaline phosphatase showed a progressive rise and skeletal bone survey did not disclose osteopenia or signs of parathyroid bone disease. A course of calcitriol failed to improve the myopathy, which responded promptly and dramatically to parathyroidectomy. Uremic parathyroid myopathy, which has a characteristic clinical picture, must be differentiated from other neuropathic or myopathic conditions that require specific treatments. Progressive parathyroid myopathy is, by itself, an indication for parathyroidectomy, which is curative in this case.

Ability of cyclosporine to promote the growth of transplanted ultraviolet radiation-induced tumors in mice.

The use of immunosuppressive agents (primarily azathioprine and prednisone) to promote human allograft survival is known to be associated with an enhanced rate of skin cancer development by ultraviolet radiation (UVR). These observations raise the question of whether the immunosuppressive agents are functioning as cocarcinogen or whether they augment UVR-induced tumors by their successive influence on normal tumor-directed immune responses. In this report we have examined the effect of cyclosporine (CsA) on the capacity of murine UVR-induced tumors to grow following their transplantation to syngeneic recipients. We found that transplanted UVR tumors, selected for their inability to grow in normal recipients, were capable of progressive growth following implantation into CsA-treated recipients. This CsA-induced tumor-susceptible state could be reversed by treatment of prospective recipients with the drug cyclophosphamide (CY), supporting the concept that CsA was functioning in vivo by its capacity to promote suppressor cell generation. Further studies established that CsA treatment needed to be given at or near the time of tumor transplantation for susceptibility to occur. Our findings support the possibility that CsA is capable of promoting the survival and progression of UVR-induced skin tumors via its capacity to enhance the dominance of suppressor-cell-controlled immune responses.

Anterior ischemic optic neuropathy as a complication of hemodialysis.

Hypotension is a common and sometimes difficult to manage occurrence during hemodialysis. The consequences of dialysis-induced hypotension are unpredictable. Anterior ischemic optic neuropathy is an entity associated with many systemic processes, including shock. Anterior ischemic optic neuropathy presents as sudden painless loss of vision. This is the first report of anterior ischemic optic neuropathy as a result of hemodialysis-associated hypotension.