1/7. A case of cholestatic autoimmune hepatitis and acute liver failure: an unusual hepatic manifestation of mixed connective tissue disease and sjogren's syndrome.Although hepatomegaly is reported to occur occasionally in patients with mixed connective tissue disease (MCTD) or sjogren's syndrome (SS), autoimmune liver diseases such as primary biliary cirrhosis, sclerosing cholangitis, and autoimmune hepatitis in association with MCTD or SS have rarely been described. We report a case of severe cholestatic autoimmune hepatitis presenting with acute liver failure in a 40-yr-old female patient suffering from MCTD and SS. The diagnosis of MCTD and SS was made at the age of 38. The patient presented severe jaundice and elevation of conjugated bilirubin. The patient denied alcohol and drug use and had no evidence of viral hepatitis. On the 8th day of her hospitalization, the patient developed grade III hepatic encephalopathy. She was diagnosed as autoimmune hepatitis presenting with acute liver failure based on clinical features, positive FANA and anti-smooth muscle antibodies, negative anti-mitochondrial antibodies, high titers of serum globulin, liver biopsy findings, and a good response to corticosteroid therapy, The patient was managed with prednisolone and the clinical symptoms, liver function test results, and liver biopsy findings showed much improvement after steroid therapy.- - - - - - - - - - ranking = 1keywords = suffering (Clic here for more details about this article) |
2/7. Co-occurrence of spondyloarthropathy and connective tissue disease: development of sjogren's syndrome and mixed connective tissue disease (MCTD) in a patient with ankylosing spondylitis.spondylarthropathies (SpA) and connective tissue diseases (CTD) are clinically distinct entities which, at first glance, seem to have little in common. However, a link between SpA and CTD has recently been suggested by a study in which a higher prevalence of sjogren's syndrome (SS) and sicca symptoms was reported in patients with ankylosing spondylitis (AS) and undifferentiated SpA (1). Another link between SpA and CTD is a possible side effect of a DMARD widely used to treat SpA: sulfasalazine (SAS). SAS was reported to induce antinuclear antibodies (ANA) and systemic lupus erythematosus (SLE)-like syndromes such as drug-induced lupus. This report describes a 54-year-old white male, HLA B27-positive AS patient with some syndesmophytes who, after 15 years of disease, developed SS with salivary gland involvement, Raynaud's syndrome and anti-Ro antibodies. Then, 20 years after the onset of AS, he became acutely ill, suffering severe myositis and myocarditis along with swollen hands and highly elevated autoantibody titers recognizing UIRNP; his condition was interpreted as mixed connective tissue disease (MCTD). The patient had been treated with SAS and azathioprine (AZA) alone several times during the last years because he had not tolerated other DMARDs. A combination of both drugs had been prescribed 3 weeks before a severe flair because of progredient high disease activity with painful peripheral arthritis of the MCP and PIP joints which, however, had not shown radiographic erosions. We describe the rare development of MCTD in an AS patient and report, for the first time, the onset of MCTD potentially triggered by sulfasalazine.- - - - - - - - - - ranking = 1keywords = suffering (Clic here for more details about this article) |
3/7. A case of mixed connective tissue disease showing a lymphoid follicle in muscle pathology.We report a 74-year-old man suffering from mixed connective tissue disease (MCTD) accompanied by a lymphoid follicle in muscle pathology. He showed proximal muscle weakness and arthritis in finger joints. His laboratory findings were characteristic of MCTD. prednisolone administration favorably improved his symptoms. A lymphoid follicle is a rare pathological finding in inflammatory myopathies. The immunohistochemical findings suggest that the lymphoid follicle would be involved in the specific role for the pathogenesis of MCTD.- - - - - - - - - - ranking = 1keywords = suffering (Clic here for more details about this article) |
4/7. Recurrent bilateral optic neuropathy in mixed connective tissue disease.Multiple attacks of optic neuropathy occurred in a young woman suffering from mixed connective tissue disease despite maintenance therapy with corticosteroids and cytotoxic agents.- - - - - - - - - - ranking = 1keywords = suffering (Clic here for more details about this article) |
5/7. mixed connective tissue disease with fatal pulmonary hypertension.An autopsy case of mixed connective tissue disease (MCTD) with pulmonary hypertension is presented. A 34-year-old woman suffering from arthralgia, Raynaud's phenomenon, and dyspnea of 6-years duration was diagnosed as having MCTD on the basis of a high titer (1:160,000) of serum antibody to the ribonuclease-sensitive component of extractable nuclear antigen. Examination of cardiac function revealed the complication of pulmonary hypertension. autopsy revealed concentric intimal cellular proliferation of the small arteries and arterioles of both lungs. Typical plexiform lesions of these vessels were also observed. These findings coincide with those of plexogenic pulmonary angiopathy of primary pulmonary hypertension (PPH). This is the second autopsy case of MCTD with fatal pulmonary hypertension reported and our observations suggest that some cases with PPH who had immunological abnormalities but could not be classified as cases of classical collagen disease, may have been induced by MCTD.- - - - - - - - - - ranking = 1keywords = suffering (Clic here for more details about this article) |
6/7. Collagen vascular disease: can behavior therapy help?This study examined the efficacy of a simple autogenic and biofeedback treatment package in the management of Raynaud's Phenomenon secondary to diagnosed collagen vascular disease. The patient, diagnosed as suffering from mixed connective tissue disease, had an average of 6.3 vasospastic attacks per day during a 2 week baseline period. The frequency of daily attacks dropped to 4.2 after 10 weeks and 2.5 attacks after 1 yr of training. In addition, the patient displayed a gradual improvement in the ability to maintain digital skin temperature in the presence of ambient cold stress.- - - - - - - - - - ranking = 1keywords = suffering (Clic here for more details about this article) |
7/7. Metaplastic bone formation in the subcutaneous nodule of a patient with mixed connective tissue disease.Cutaneous ossification is a rare phenomenon in collagen diseases, despite the rather frequent occurrence in these diseases of dystrophic calcinosis. We observed metaplastic woven bone formation associated with calcification in biopsy material obtained from a 49-year-old woman suffering from mixed connective tissue disease together with multiple subcutaneous indurations. This is the first case of the presence of metaplastic bone formation in a patient with mixed connective tissue disease.- - - - - - - - - - ranking = 1keywords = suffering (Clic here for more details about this article) |