title: [Is mixed connective tissue disease a distinct rheumatic disease].
abstract: Mixed connective tissue disease (MCTD) as a distinct clinical entity is still controversial. To address the issue, the authors observed the evolution of 50 patients diagnosed as MCTD at our clinic, evaluated the reliability of three different diagnostic criteria proposed by Sharp, Alarcon-Segovia and Kasukawa respectively and performed HLA-A, -B and -DR antigen typing in 38 of the patients. The results showed that: (1) 13 (26.9%) of the 50 MCTD patients subsequently developed other connective tissue disease (OCTD) including 7 systemic lupus erythematosus (SLE), and 6 progressive systemic scleroderma (PSS). (2) among 23 of the MCTD patients fulfilling Sharp's criteria, 1 (4.3%) developed PSS, but among 23 of the patients fulfilling Kasukawa's, not Sharp's, 7 (30.4%) developed OCTD and among 27 of the patients fulfilling Alarcon-Segovia's, not Sharp's, 12 (44.4%) developed OCTD. (3) in the frequencies of DR, and DR5, there were significant differences between patients fulfilling Sharp's (60.9%, 56.5%) and the normal controls (24.3%, P < 0.005, RR = 4.7 and 21.4%, P < 0.005, RR = 4.6%), but no significant differences between the patients not fulfilling Sharp's and normal controls (P > 0.05). We concluded that: (1) MCTD is a distinct rheumatic disease. (2) Sharp's criteria is the most reliable for diagnosis of MCTD.
authors.affiliation: Department of Rheumatology and Clinical Immunology, Renji Hospital, Shanghai.
authors.forename: S
authors.initials: S
authors.lastname: Chen
authors.forename: N
authors.initials: N
authors.lastname: Shen
authors.forename: H
authors.initials: H
authors.lastname: Yang
mesh_terms: D000293:Adolescent; D000328:Adult; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D015804:HLA-DR4 Antigen / Q000032:analysis; D015805:HLA-DR5 Antigen / Q000032:analysis; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000175:diagnosis; D008297:Male; D008875:Middle Aged; D008947:Mixed Connective Tissue Disease / Q000175:diagnosis* / Q000276:immunology; D012015:Reference Standards; D012595:Scleroderma, Systemic / Q000175:diagnosis
chemical_list: D015804:HLA-DR4 Antigen; D015805:HLA-DR5 Antigen