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.
Adult ; Cholestasis/*etiology ; Female ; Hepatitis, Autoimmune/*etiology ; Human ; Liver Failure, Acute/*etiology ; Mixed Connective Tissue Disease/*complications ; Sjogren's Syndrome/*complications

Parasymphyseal insufficiency fractures are uncommon. Furthermore, none have been reported in systemic rheumatic diseases other than rheumatoid arthritis. In this article we report on parasymphyseal insufficiency fractures in a patient with mixed connective tissue disease.
Adult ; Case Report ; Female ; Fractures, Stress/etiology+ACo- ; Human ; Mixed Connective Tissue Disease/complications+ACo- ; Pubic Bone/injuries+ACo-

PURPOSE: To evaluate the long-term surgical results of 120 endoscopic conjunctivodacryocystorhinostomy (CDCR) procedures using a porous polyethylene (MEDPOR(R)) coated tear drain (MCTD(R)). METHODS: From 2002 to 2007, 120 patients who had been treated with endoscopic CDCR using MCTD(R) were investigated for its success rates and complications. After an osteotomy was made under nasal endoscopy, a tunnel was created from the caruncle in the conjunctival sac to the nasal cavity through the newly created ostium, and then the tunnel was enlarged to allow the insertion of the MCTD(R) The length of the tube to be inserted was determined under endoscopic examination. An anchoring suture was placed at the medial canthus to both the conjunctiva and the skin using a 5-0 Vicryl suture in the fashion of a purse string suture. RESULTS: Causes of obstruction included failed dacryocystorhinostomy (DCR) (74 cases) and idiopathic obstruction (22 cases). Postoperative complications were encountered in four cases with tube loss, 21 cases with a buried tube, 8 cases of extrusion to the conjunctival side, and 11 cases with obstruction caused by conjunctival incarceration or granuloma. The postoperative success rate was 89.1%. CONCLUSIONS: The authors concluded that endoscopic CDCR using MCTD(R) is an alternative to the standard conventional method for preventing dislodgement of the tube postoperatively. However, surgeons should consider that it may be challenging to insert the MCTD(R) and that in cases requiring removal, the tube can be difficult to remove due to adhesions.
Conjunctiva ; Dacryocystorhinostomy ; Endoscopy ; Granuloma ; Humans ; Mixed Connective Tissue Disease ; Nasal Cavity ; Osteotomy ; Polyethylene ; Polyglactin 910 ; Postoperative Complications ; Skin ; Sutures

The concurrence of ankylosing spondylitis (AS) in a patient with mixed connective tissue disease (MCTD) is rarely described in the literature. Significant and sustained efficacy with tumor necrosis factor (TNF)-alpha blockers has been demonstrated in AS patients. However, evidence to date has revealed associated side effects, including antinuclear antibody induction and development of a lupus-like syndrome. Several authors have reported lupus-like manifestations in MCTD patients treated with TNF-alpha blockers used to control peripheral polyarthritis. In our case report, we demonstrate a good response to etanercept therapy for refractory sacroiliitis in a patient with coexisting AS and MCTD, without development of a lupus-like syndrome. This demonstrates that etanercept therapy may be an appropriate therapeutic agent for sacroiliitis in MCTD patients, as it is in AS alone.
Female ; Humans ; Immunoglobulin G/*therapeutic use ; Magnetic Resonance Imaging ; Middle Aged ; Mixed Connective Tissue Disease/complications/*drug therapy/*pathology ; Receptors, Tumor Necrosis Factor/*therapeutic use ; Sacroiliac Joint/*drug effects/*pathology ; Spondylitis, Ankylosing/complications/*drug therapy/*pathology ; Treatment Outcome

A case of Mycobacterium avium arthritis in a 39-year-old female patient with mixed connective tissue disease (MCTD) was reported. An extra-articular abscess had formed outside the knee joint and extended down the calf. A culture was taken of the abscess and synovial fluid disclosed Mycobacteriun avium. This was resistant to most anti-tuberculosis agents. A combination of anti-tuberculosis drugs followed a total resection of the abscess. We concluded that M avium septic arthritis could insidiously develop into an extra-articular abscess. A combination of anti-tuberculosis drugs with a total resection of the abscess was an effective treatment.
Adult ; Antitubercular Agents/therapeutic use ; Arthritis, Infectious/*microbiology/therapy ; Female ; Human ; Knee Joint/*microbiology/surgery ; Mixed Connective Tissue Disease/*complications ; Mycobacterium avium/drug effects/*isolation & purification ; Mycobacterium avium-intracellulare Infection/*complications/therapy