Analysis of clinical features and the outcome in 91 cases of mixed connective tissue diseases

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1 mixed connective tissue disease MCTD MCTD MCTD antinuclear antibodies ANA ribosenuclear protein antibodies RNP MCTD 1 ~ 6 8 systemiclupuserythematosus SLE 2 anti-neutrophil cytoplasmic antibodyes ANCA ANCA-associated vasculitis AAV 2 rheumatoid arthritis RA 1 primary Sj gren s syndrome pss 50 MCTD SLE MCTD R doi /j. issn X A X Analysis of clinical features and the outcome in 91 cases of mixed connective tissue diseases SHI Yu-hong 1 2 LI Ru 1 CHEN Shi 1 SU Yin 1 JIA Yuan 1 1. Department of Rheumatology and Immunology Peking University People s Hospital Beijing China 2. Department of Rheumatology and Immunology Guilin Medical University Guilin China ABSTRACT Objective To investigate the clinical features and prognosis of mixed connective tissue disease MCTD. Methods Clinical laboratory and instrumental examination information of 91 patients with MCTD who were diagnosed between 1990 to 2008 in Peking University People s Hospital were collected and analyzed retrospectively. These patients were following-up and different outcoms compared. Results The most common manifestations of MCTD patients were Raynaud phenomenon arthralgia arthritis fever acratia positivities of antinuclear antibodies anti-ana and ribosenuclear protein antibodies anti-rnp which were 94. 5% 78% 46. 2% 48. 4% 53. 9% 100% and 100% respectively. Six patients died and 22 patients were lost in the follow-up after discharge. Among the remaining 63 patients 8 developed into systemic lupus erythomatosus SLE and 2 into antineutrophil cytoplasmic antibodies-associated vasculitis AAV 1 into primary Sj gren s syndrome pss and 2 into rheumatoid arthritis RA at one to six years after diagnosis of MCTD. The patients who initially manifested as alopecia proteinuria thrombocytopenia low complement were more likely to develop into SLE. Conclusion MCTD can develop into various autoimmune diseases such as SLE pss RA AAV. Some clinical features can probably predict future outcomes. KEY WORDS Mixed connective tissue disease Pathological conditions signs and symptoms Tueatment outcome Follow-up studies mixed connective tissue disease systemic sclerosis SSc systemiclupuserythematosus SLE rheumatoid arthritis RA polymyositis MCTD antinuclear antibody ANA u1-ribosenuclear protein antibodies u1-rnp PM / SLE RA dermatomyositis DM SSc MCTD Corresponding author s jiayuan1023@ sina. com http / /www. cnki. net /kcms /detail / R html

2 MCTD MCTD MCTD MCTD Kasukawa ~ ± ± ± ± / ANA DNA dsdna extractable nucler antigen ENA u1- RNP anticardiolipin antibodies ACL rheumatoid factor RF X CT ~ SPSS x 珋 ± s P < MCTD ANA u1-rnp Table 1 1 Clinical features of MCTD patients in first diagnosis Clinical features n % Raynaud phenomenon Arthralgia Fatigue Fever Rash Arthritis Dry mouth Swollen hands Myalgia Dry eyes Hair loss Oral ulcers Serous effusions Photoallergy Parotid enlargement Interstitial lung disease Pulmonary hypertension Table 2 2 Libratory features of MCTD patients in first diagnosis Libratory features n % Leukopenia Anemia Thrombocytopenia Proteinuria Hematuria Leukocyte in urine CK Hyperlipidemia Hypergammaglobulinemia Hypocomplementemia ANA u1-rnp SSA SSB dsdna RF ACL CK creatine kinase.

3 ± SLE MCTD SLE 2 MCTD anti-neutrophil cytoplasmic antibodyes ANCA ANCA-associated vas- SLE MCTD 3 MCTD culitis AAV 2 RA 1 primary Sj gren s syndrome pss 50 MCTD h 0. 5 g 3 SLE SLE ~ ~ ~ 3 AAV 2 3 ~ 6 1 ~ 24 6 RA 1 1 pss MCTD 3 SLE pss RA AAV MCTD n % Table 3 The comparison for clinical features of MCTD with different outcome n % Clinical features SLE n = 8 AAV n = 2 RA n = 2 pss n = 1 MCTD n = 50 Raynaud phenomenon Fever Arthralgia Arthritis Rash Swollen hands Hair loss # Photoallergy Myalgia Fatigue Oral ulcers Dry mouth Dry eyes Parotid enlargement Serous effusions # P < compared with MCTD group. 4 SLE pss RA AASV MCTD n % Table 4 The comparison for lobratory features of MCTD with different outcome n % Clinical features SLE n = 8 AAV n = 2 RA n = 2 pss n = 1 MCTD n = 50 Leukopenia Anemia Thrombocytopenia # Hypergammaglobulinemia Hypocomplementemia # CK Proteinuria SSA SSB ANA # P < P < compared with MCTD group.

4 / MCTD 19 MCTD SSc SLE ANA u1-rnp MCTD MCTD Bodolay 7 MCTD 5 MCTD 96. 4% % ~ % SLE RA AAV 42. 8% ~ 66. 6% 6 8 CT SLE 8 Sharp % 34. 1% Alarcon-Segovia 10 Kahn 11 X SLE SLE SLE 19 MCTD 2009 SLE Systemic Lupus International Collaborating Clinic SLICC Sharp 2 30 SLE 20 3 MCTD Sharp 2 Alarcon- Segovia 10 Kahn 11 Kasukawa 1 SLE MCTD SSc 4 SSc 4 4 u1-rnp RA SLE RA pss MCTD SSc PM / DM RA 12 u1-70kdsnrnp MCTD Sharp MCTD u1-rnp MCTD MCTD SLE SSc MCTD ~ ± MCTD 4 HLA-DR4 HLA-DR5 3 SLE 1 ~ 6 1

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