Japanese Journal of Clinical Immunology
Online ISSN : 1349-7413
Print ISSN : 0911-4300
ISSN-L : 0911-4300
Undifferentiated connective tissue syndromes (UCTS) accompanied by laryngeal involvement and autoimmune hepatitis
Makoto NishinaritaShuji OhtaMasashi UesatoYuji OkaToshiro KamoshidaAtsushi Takahashi
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1995 Volume 18 Issue 5 Pages 559-565

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Abstract
Here we report a patient with undifferentiated connective tissue syndromes (UCTS) who developed hoarseness during exacerbation of autoimmune hepatitis. A 51-year-old woman was hospitalized in November 1993 because of hoarseness and liver dysfunction. She had demonstrated Raynaud's phenomenon, polyarthralgia and hoarseness scince 1992. In August 1993, liver dysfunction was noted.
On admission, laboratory data showed mild leukopenia, thrombocytopenia (WBC 3, 900/mm3, platelete 12.4×104/mm3), and elevations of transaminase (GOT 96 IU/l, GPT 79 IU/l) and IgG (4, 556mg/dl).
Anti-nuclear antibody (ANA) and anti-smooth muscle antibody were positive. Other autoantibodies including anti-DNA antibody, anti-Scl 70 antibody were all negative. LE test and LE cells were also negative.
On laryngoscopic examination, lesions that appeared similar to a bamboo-joint were noted at the middle of the bilateral vocal cords. Pathological findings of liver biopsy specimen were compatible with autoimmune hepatitis.
She was treated with 30mg of prednisolone. Polyarthralgia, hoarseness and the abnormalities of the transaminase levels improved rapidly. Laryngoscopic findings were also normalized.
We concidered this laryngeal involvement to be acute laryngitis accompanied by some UCTS, including a typical systemic lupus erythematosus (SLE) because of arthritis, cytopenia and ANA positivity. Involvement of the larynx in collagen disease is rarely mentioned in published reports.
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© The Japan Society for Clinical Immunology
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