Cases reported "Scleroderma, Limited"

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1/6. Bilateral primary orbital non-Hodgkin's lymphoma in a patient with scleroderma: a case report.

    Orbital presentation of non-Hodgkin's lymphoma (NHL) is uncommon but occurs both as the only site of disease and as a site of recurrence. Primary orbital NHLs are usually low-grade, and mostly extranodal marginal zone/mucosa associated lymphoid tissue B-cell neoplasms. They are commonly associated with chronic inflammatory conditions. The issue of bilaterality in orbital lymphoma is not very common. The onset of malignant lymphoproliferation may precede, follow, or exist simultaneously with scleroderma. Different treatment modalities were applied in orbital NHL such as surgery, radiation therapy, chemotherapy or both. We report a 57-year-old man with scleroderma presenting with eyelid hernias who had biopsy-proven marginal zone NHL, successfully treated with radiotherapy and combined chemotherapy, and also review the literature. ( info)

2/6. association of limited scleroderma and pulmonary hypertension in a patient with primary biliary cirrhosis.

    We report a male patient with primary biliary cirrhosis (PBC) who developed limited scleroderma (l-SSc) and pulmonary hypertension (PHT). He had noticed shortness of breath seven months earlier, which slowly progressed before admission. Sclerodactyly and telagiectasia of the fingers and chest wall were found. Chest X-ray and Doppler echocardiography suggested the presence of PHT. Histologic examination of the liver (needle biopsy) revealed stage two PBC, and histologic findings of the skin (obtained from the dorsum of right finger IV) were compatible with l-SSc. Direct measurement of pulmonary arterial pressure revealed PHT with normal capillary wedge pressure during right heart catheterization. A striking increment of plasma thromboxane B(2) across the lungs was found, which suggested that thromboxane A(2) (precursor of thromboxane B(2)) contributed considerably to a rise in pulmonary vascular resistance leading to PHT. ( info)

3/6. The development of connective tissue diseases in patients with autoimmune hepatitis: a case series.

    OBJECTIVES: To study the prevalence of connective tissue diseases (CTD) in patients with autoimmune hepatitis (AIH). methods: We identified 11 cases of AIH over the past 7 years at our institution, through a systematic chart review of patients with this diagnosis. Their charts were reviewed for the development of systemic CTD. RESULTS: Three of the 11 patients with a definitive diagnosis of AIH developed systemic CTD. All were white: 2 women and 1 man, with an age range of 33 to 62 years, and with disease duration of 1 to 7 years. One patient developed systemic lupus erythematosus (SLE) with vasculitis and peripheral neuropathy. The second developed limited scleroderma and the third developed undifferentiated connective tissue disease (UCTD) and interstitial lung disease. There appear to be shared susceptibility alleles for AIH and CTD in addition to the shared positive autoantibodies. CONCLUSIONS: patients with AIH may be at increased risk for developing systemic CTD. Conversely, a review of the literature reveals that patients with systemic CTD may be at increased risk of developing AIH. patients with either AIH or CTD should be monitored for further development of concurrent autoimmune diseases. ( info)

4/6. Ischemic scleroderma wounds successfully treated with hyperbaric oxygen therapy.

    Hyperbaric oxygen therapy (HBOT) has been used to treat refractory wounds for the last several decades, with the majority of research focusing on wounds secondary to arterial insufficiency. We describe 2 patients with scleroderma with intractable bilateral extremity ulcers. Local ischemia was identified using transcutaneous oximetry. Each patient then underwent 30 treatments of HBOT at a relative depth of 2.4 ATA with resulting wound healing. This is the first reported successful use of HBOT to treat scleroderma ulcers, and may represent an unrecognized treatment option for these notoriously difficult chronic wounds. ( info)

5/6. pulmonary veno-occlusive disease and scleroderma associated pulmonary hypertension.

    OBJECTIVE: pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary hypertension (PH). We describe a series of 4 patients with systemic sclerosis (SSc), concomitant PH, and biopsy-proven/presumed PVOD. We review the literature describing the association of PVOD and SSc and discuss diagnostic features and treatment implications. In our case series, treatment with an endothelin receptor antagonist did not confer a beneficial treatment effect. ( info)

6/6. Scleroderma renal crisis in a patient with anticentromere antibody-positive limited cutaneous systemic sclerosis.

    We have encountered a 68-year-old Japanese woman with limited cutaneous systemic sclerosis who developed de novo onset of accelerated hypertension and renal dysfunction; thus we diagnosed scleroderma renal crisis. Anticentromere antibody alone was identified, and not anti-dna topoisomerase I antibody, anti-rna polymerase antibodies, anti-Th/To antibodies, or antiribonucleoprotein antibodies, even with use of immunoprecipitation assay. She was successfully treated with angiotensin-converting enzyme inhibitor. This case, scleroderma renal crisis with detection of anticentromere antibody, is thought to be extremely uncommon. ( info)

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