Treatment of acute c-ANCA–positive vasculitis with mycophenolate mofetil

Acute cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA)–positive vasculitis is usually treated with cyclophosphamide and corticosteroids. The incidence of cyclophosphamide-induced lung injury, a potentially life-threatening event, is about 1%. We report on a patient with a history of cyclophosphamide-induced lung injury 2 months after initial treatment of systemic c-ANCA–positive vasculitis. Six months later, the patient presented with acute renal failure caused by an acute relapse of vasculitis. Mycophenolate mofetil (MMF) is a potent immunosuppressive drug that recently has been shown to be effective in the maintenance therapy of c-ANCA–positive systemic vasculitis. With the patient's informed consent, we started treatment with MMF in combination with corticosteroids. Subsequently, anti-proteinase-3-titer (anti-Pr3-titer) returned to normal and renal function improved. In conclusion, MMF in combination with corticosteroids may be useful in the treatment of acute c-ANCA–positive vasculitis.     

Refractory Hypertension and Anemia in End-Stage Renal Disease: An Unusual Manifestation of Kimura's Disease

Hypertension and anemia are common in uremic patients. This article describes a 35-year-old uremic Taiwanese who was admitted to our hospital with refractory hypertension and refractory anemia following chronic hemodialysis for more than two years. He was diagnosed with Kimura's disease finally. Refractory hypertension and refractory anemia were noted over two years before an enlarged inguinal lymph node was observed. The symptoms lead to the diagnosis of Kimura's disease. Unlike most cases, refractory hypertension and refractory anemia were first noted before the inguinal mass and eosinophilia were presented. The inflammatory parameters increased when the disease was active. Steroid treatment was conducted, and the symptoms including hypertension and anemia promptly decreased. To the authors' knowledge, this case is for first one in which Kimura's disease has induced refractory hypertension and anemia in an ESRD patient and in which these symptoms rapidly subsided following steroid treatment. The activity of Kimura's disease is closely related refractory hypertension, suggesting that inflammation may be involved in refractory hypertension and anemia in a dialytic patient with Kimura's disease.     

Transformation from Tubulointerstitial Nephritis to Crescentic Glomerulonephritis: An Unusual Presentation of ANCA-Associated Renal Vasculitis

A 44-year-old man with acute renal failure and antineutrophil cytoplasmic antibodies (ANCA) positivity was described. The first renal biopsy specimen showed tubulointerstitial nephritis (TIN) with normal glomeruli. However, delayed recovery of renal function with low-dose steroid treatment for TIN prompted a second renal biopsy 1 month later; and the specimen demonstrated a dramatically different morphology, with necrotizing and crescentic glomerulonephritis. Improvement in renal function occurred, together with reduction of ANCA titers, following intensive immunosuppressive therapy. This case illustrates an unusual presentation of TIN in ANCA-associated renal vasculitis. The possible pathogenetic mechanism are discussed.     

Misdiagnosis of Addison's Disease in a Patient with End-Stage Renal Disease

Addison's disease is a rare disorder in patients with end-stage renal disease (ESRD). In patients, the diagnosis of Addison's disease is difficult in clinical practice because most of the clinical findings of this disease are similar to those of the renal failure. We present a 51-year-old male patient, who underwent hemodialysis therapy for 8 years, diagnosed with Addison's disease after having myalgia, skin hyperpigmentation, weight loss, sweating, and nausea for the past few weeks. The physical examination was completely normal except for muscle weakness, hyperpigmentation on labial mucosa and skin in a patient. The laboratory tests revealed anemia and hypoglycemia. Serum cortisol, adrenocorticotropic hormone (ACTH) levels, and ACTH stimulation test results were consistent with Addison's disease. Adrenal computerized tomography revealed bilateral atrophic glands. Additionally, it was found that elevated serum thyroid stimulating hormone levels and antithyroid peroxidase antibody titer were positive. Our purpose is to emphasize that physicians should be alert to the potential for additional different conditions particularly in terms of adrenal failure in patients with ESRD.     

Pulmonary Renal Syndrome Due to Anti-GBM and IgM C-ANCA Disease Requiring the Use of Novel Therapeutic Agents

There are several known causes for the clinical syndrome of pulmonary hemorrhage and acute renal failure. Here, we report a unique case of a 50-year-old man presenting in this manner. The initial diagnosis was one of antiglomerular basement membrane (anti-GBM) disease that responded well to steroids, cyclophosphamide, and plasma exchange (PE). The pulmonary hemorrhage resolved, but he remained dialysis dependent. However, despite falling anti-GBM titers, the symptoms relapsed and standard therapy was reinitiated with limited success. The anti-GBM antibody titer fell to zero despite clinical deterioration, prompting a search for an alternative diagnosis. He was found to be IgM anti-proteinase-3 antineutrophil cytoplasmic antibody (C-ANCA) positive. The pulmonary hemorrhage responded successfully to the use of intravenous immunoglobulin and the antilymphocyte monoclonal antibody CD52. To our knowledge, this is the first known case of IgM C-ANCA in association with anti-GBM disease. As such, it highlights the predominance of pulmonary hemorrhage in this condition, as well as the need to consider alternative therapies in refractory cases.     

Cost-Effectiveness of Lung Transplantation in Relation to Type of End-Stage Pulmonary Disease

The purpose of this study was to explore the relationship between diagnosis and the cost-effectiveness and cost-utility of lung transplantation. A microsimulation model was used, based on empirical data from the Dutch lung transplantation program, collected between 1991 and 1999. We assessed life-years, quality-adjusted life-years, and costs with and without transplantation for the diagnostic categories alfa-1 antitrypsin deficiency, COPD/emphysema, bronchiectasis, primary and secondary pulmonary hypertension, cystic fibrosis, and pulmonary fibrosis. Alfa-1 antitrypsin deficiency and bronchiectasis had the highest survival gain. Secondary pulmonary hypertension and pulmonary fibrosis had the lowest survival gain and the lowest gain of quality-adjusted life-years. As compared with COPD/emphysema, alfa-1 antitrypsin deficiency, bronchiectasis, and CF had 25%, 40% and 19% more favorable cost-effectiveness ratios, respectively. Cost-utility ratios varied less, with values of -7%, -14% and -11% for alfa-1 antitrypsin deficiency, bronchiectasis, and primary pulmonary hypertension, respectively, compared with COPD. In conclusion, our model suggests that there is considerable variation in cost-effectiveness and, to a lesser degree, in cost-utility between the different diagnostic categories. These variations are the result of differences in survival and in quality of life with and without lung transplantation.     

The Effectiveness of Diclofenac Sodium in the Treatment of Mondor's Disease of the Breast: The Topical Patch Compared to the Oral Capsules

Mondor's disease of the breast is a rare, benign sclerosing superficial thrombophlebitis of the subcutaneous veins of the anterior or lateral chest wall, which is treated conservatively. We aim in this study to evaluate the outcome and effectiveness of our treatment protocol using oral diclofenac sodium and topical diclofenac sodium patch in 172 patients. A retrospective database analysis of 172 female patients between January 2001 and December 2010 was done. The treatment protocol consisted of group 1: treatment by oral diclofenac sodium 100 mg once daily for 3 weeks. Group 2: treatment by diclofenac sodium patches for 8 hours twice daily (morning and evening) for 1 week. The patients were instructed to document the time as soon as pain relief is achieved following the patch application and the intake of the oral dose. The incidence rate was 2.49%. Diclofenac sodium patch was statistically found to be significantly better in subsiding the inflammatory process of the veins, relieving the pain, and enhancing faster healing rate. We conclude that diclofenac sodium patch showed a promising role in the treatment of Mondor's disease of the breast by significantly decreasing the inflammatory process due to its transdermal migration action within a short period and the ability to reach a high local concentration . It achieved the best results for rapid relief of pain and disease regression compared to the oral capsules. Therefore, our protocol was changed to implement diclofenac sodium patch as the first choice in treating Mondor's disease of the breast.