1/26. A case of basal cell carcinoma of the skin in a patient with systemic sclerosis.A 46-year-old man complained of pain and stiffness in both hands. Most of his fingertips were shortened, and Raynaud's phenomenon was evident. The skin on his face and hands was hard and taut. An ulcerating mass measuring 3.2 x 2.5 cm was found on his left infraorbital area. biopsy revealed basal cell carcinoma. Antinuclear antibody and anti-topoisomerase I antibody were strongly positive. A high-resolution computed tomography (CT) scan of the lungs revealed interstitial fibrosis. Esophageal manometry showed low lower esophageal sphincter pressure and gastroesophageal reflux. Under the diagnosis of basal cell carcinoma accompanying systemic sclerosis (SSc), surgical excision of the mass was performed, and immunosuppressant and vasodilator therapies were conducted.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
2/26. Cardiac death after autologous stem cell transplantation (ASCT) for treatment of systemic sclerosis (SSc): no evidence for cyclophosphamide-induced cardiomyopathy.In patients with systemic sclerosis (SSc) treatment-related mortality after autologous stem cell transplantation (ASCT) appears to be increased as compared to patients with hematological malignancies. In our phase I/II study on ASCT in autoimmune diseases a patient with SSc died on day 2 after ASCT. Here we report the results of the autopsy which revealed advanced pulmonary and cardiac fibrosis as the most probable cause of death. In spite of detailed technical examination before enrollment, the cardiopulmonary function tests did not reflect the advanced stage of the disease. We conclude that in selected patients with SSc, biopsies should be performed to reduce mortality after ASCT.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
3/26. pulmonary fibrosis induced by cyclophosphamide.OBJECTIVE: To report a case of pulmonary fibrosis resulting from use of cyclophosphamide as chemotherapy to treat a patient with breast cancer. CASE SUMMARY: We describe the case of a 52-year-old woman with breast cancer who developed pulmonary fibrosis after four cycles of chemotherapy that included cyclophosphamide. Pulmonary function tests revealed the presence of a severe ventilatory restriction. The open lung biopsy revealed pulmonary fibrosis with vascular sclerosis and signs of pulmonary hypertension. DISCUSSION: cyclophosphamide is an alkylating agent that has been associated with interstitial pneumonia and pulmonary fibrosis. The frequency of these unwanted effects is <1%. The clinical picture consists of the progressive appearance of dyspnea and a non-productive cough that progresses to severe pulmonary insufficiency. The risk factors described for these complications have been the use of chemotherapy regimens that include other drugs with known pulmonary toxicities, the cumulative total dose, the addition of radiotherapy, and the use of high doses of cyclophosphamide. CONCLUSIONS: Even though the frequency of pulmonary fibrosis in patients treated with cyclophosphamide-based chemotherapy regimens is low, the presence of dyspnea and an interstitial pattern in a patient makes it necessary to consider that possible drug toxicity. The open lung biopsy is the most accurate diagnostic technique for these cases. The discontinuation of cyclophosphamide and treatment with corticosteroids is usually followed by clinical recovery in approximately 50% of patients and, in some cases, reversal of the lung injury.- - - - - - - - - - ranking = 0.2keywords = sclerosis (Clic here for more details about this article) |
4/26. Systemic sclerosis complicated by diffuse alveolar hemorrhage.A 38-year-old woman with limited cutaneous systemic sclerosis and pulmonary fibrosis developed diffuse alveolar hemorrhage during the course of her disease that responded well to steroids. We present the clinical history of the patient and discuss the different theories behind the association. The importance of steroid therapy for treatment of alveolar hemorrhage in this particular condition is emphasized.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
5/26. dermatofibrosarcoma protuberans with lung metastasis in a patient with progressive systemic sclerosis.A female patient with progressive systemic sclerosis and pulmonary fibrosis had a dermatofibrosarcoma protuberans on the right thigh. After resection of the tumor, new lesions occurred in the scar, and wide excision was repeated. Two years later, a lung metastasis was discovered, and segmental resection was done. After 1 year of follow-up, no local recurrences or metastases were found.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
6/26. Incomplete septal cirrhosis associated with Wegener's granulomatosis.Incomplete septal cirrhosis, which is included in the spectrum of hepatoportal sclerosis, is characterized by parenchymal nodularity, incomplete fibrous septa, clustered or dispersed portal tract remnants, and abnormal spacing of portal tracts and hepatic veins. Hepatoportal sclerosis is known to be associated with collagen vascular diseases. Here, we describe a 73 year-old-female with incomplete septal cirrhosis. At 57 years, she presented with respiratory symptoms, and lung biopsy disclosed active arteritis with granuloma. Perinuclear antineutrophilic cytoplasmic antibody was also positive. Immunosuppressive therapy was done under the diagnosis of Wegener's granulomatosis. At 63 years, liver dysfunction was noted, and laparoscopy revealed uneven surface of the liver and dilatation of the umbilical vein. liver dysfunction progressed, and she developed encephalopathy and massive ascites. She died of sepsis at 73 years. At autopsy the liver (700 g) was macronodular with several deep depressions. The parenchyma showed fine and diffuse nodularity. Grossly visible portal and hepatic veins were patent. The above-mentioned histologic features characterizing incomplete septal cirrhosis were found. This is the first report of incomplete septal cirrhosis associated with Wegener's granulomatosis implying that vascular and extravascular lesions of Wegener's granulomatosis might have been related to the pathogenesis of incomplete septal cirrhosis.- - - - - - - - - - ranking = 0.4keywords = sclerosis (Clic here for more details about this article) |
7/26. A successful therapy with plasma exchange for interstitial pneumonia of progressive systemic sclerosis.A 47-year-old woman who was diagnosed as progressive systemic sclerosis (PSS) had acute and severe interstitial pneumonia. Based on the results of her chest roentgenogram, computed tomography, transbronchial lung biopsy (TBLB) and bronchoalveolar lavage (BAL), her interstitial pneumonia was considered to be atypical of PSS. Although she was treated with corticosteroid, methylprednisolone pulse therapy and immunosuppressive drug, the effect of these drugs was insufficient as treatment for the interstitial pneumonia. Therefore, plasma exchange was attempted. After plasma exchange was carried out for three days, her symptoms improved as well as the laboratory data and chest roentgenogram without any severe side effects. We recommend plasma exchange for interstitial pneumonia of PSS as an effective treatment.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
8/26. Systemic sclerosis sine scleroderma presenting with vitiligo-like depigmentation and interstitial pulmonary fibrosis.We report a case of systemic sclerosis sine scleroderma in which vitiligo-like depigmentation and early interstitial pulmonary fibrosis predominated. The pigmentary changes occurring in systemic sclerosis are reviewed and the distinctive features of the case discussed.- - - - - - - - - - ranking = 1.2keywords = sclerosis (Clic here for more details about this article) |
9/26. Lung involvement in systemic sclerosis sine scleroderma treated by plasma exchange.Systemic sclerosis sine scleroderma can present in some patients as pulmonary interstitial fibrosis. Until now ten cases with this particular clinical variant, all men, have been reported in the literature. The knowledge of systemic sclerosis sine scleroderma presenting as lung interstitial involvement is important in clinical practice for an early diagnosis and correct therapeutic strategy. This work reports the clinico-serological features of two further cases, one a woman, of systemic sclerosis sine scleroderma with prevalent lung involvement, and describes the effects of therapeutic plasma exchange.- - - - - - - - - - ranking = 1.4keywords = sclerosis (Clic here for more details about this article) |
10/26. Focal segmental glomerulosclerosis in desquamative interstitial pneumonia.Renal involvement in desquamative interstitial pneumonitis (DIP) manifesting as chronic renal failure has been reported only once. An uncommon disorder in children, DIP has been associated with a variety of systemic disorders and has an immune-mediated pathogenesis. A 16-year-old Black male was diagnosed to have DIP on lung biopsy at the age of 10 months. He was first noted to have proteinuria at age 5 which progressed to nephrotic syndrome by age 13 when the laboratory tests showed elevated IgG, normal serum complement, increased circulating immune complexes and absent anti-GBM antibodies. A percutaneous renal biopsy specimen performed at age 13 revealed focal segmental glomerulosclerosis. Despite prednisone treatment of 2 mg/kg/day for 12 weeks, renal failure progressed requiring hemodialysis. Pulmonary functions, although reduced, remained stable.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
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