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Mixed connective tissue disease: the King Faisal Specialist Hospital experience.
MedLine Citation:
PMID:  17259766     Owner:  NLM     Status:  PubMed-not-MEDLINE    
Abstract/OtherAbstract:
BACKGROUND: The aim of this study was to assess the clinical presentation, complications and serological analysis of mixed connective tissue disease (MCTD) at King Faisal Specialist Hospital & Research Centre (KFSH&RC), and to determine the long-term clinical and immunologic outcomes. PATIENTS AND METHODS: This was a retrospective study with prospective follow-up of 18 patients with MCTD who were followed at KFSH&RC between 1982 and 1999. RESULTS: The age at onset of the disease ranged from 6 to 44 years, with a mean age of 17.9 years. The female to male ratio was 2.5:1, and the mean follow-up time was 5 years. The most frequent presenting symptoms were arthralgia in all patients, Raynaud's phenomenon in 16 patients (88%) and swollen hands in 11 patients (61%). Arthritis was seen in 12 patients (67%) and definite myositis in 10 patients (58%). The most common skin rashes encountered included lupus-like rash in 8 patients (44%), scleroderma-like rash in 8 patients (44%), and cutaneous vasculitis in 5 patients (28%). Pulmonary hypertension occurred in 4 patients (22%). Other clinical manifestations encountered were esophageal hypomotility in 10 patients (56%), myocarditis in 2 patients (11%) and proteinuria in 2 patients (11%), while various neurological manifestations were present in 7 patients (39%). All patients exhibited high titer of ANA and anti-nRNP antibodies. Five of the 18 patients (28%) had marked reduction in the anti-nRNP during remission. Following treatment, features of inflammation as well as Raynaud's phenomenon and esophageal hypomotility diminished, while pulmonary hypertension persisted. A favorable outcome was observed in 12 patients (67%); 3 patients (17%) had continued active disease, while 3 patients (17%) died, with death related to pulmonary hypertension occurring in two patients (11%). CONCLUSION: The studied patients demonstrated the typical clinical and serological findings of MCTD, which support the correlation between anti-nRNP antibody specificities and MCTD. Autoantibody reactivity against nRNP polypeptides tends to regress during prolonged disease remission. The majority of our patients had favorable outcomes, with pulmonary hypertension being the most frequent disease-associated cause of morbidity and mortality.
Authors:
Hanan Al Rayes; Ahmed Al-Sheikh; Abdullah Al Dalaan; Salman Al Saleh
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Annals of Saudi medicine     Volume:  22     ISSN:  0256-4947     ISO Abbreviation:  Ann Saudi Med     Publication Date:    2002 Jan-Mar
Date Detail:
Created Date:  2007-01-29     Completed Date:  2007-06-08     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8507355     Medline TA:  Ann Saudi Med     Country:  Saudi Arabia    
Other Details:
Languages:  eng     Pagination:  43-6     Citation Subset:  -    
Affiliation:
Department of Medicine, Section of Rheumatology, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.
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