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Published byGrace Waters Modified over 9 years ago
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Good Morning ! October 3 rd, 2011
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An overlap syndrome associated with anti-U1-RNP (ribonucleic protein) antibodies with features of SLE, scleroderma, and polymyositis In early stages, cannot be differentiated from the other connective tissue diseases (SLE, Scl, PM, DM, RA, and Sjogren’s)
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Early symptoms › Easy fatigability › Poorly defined myalgias › Arthralgias › Raynaud phenomenon
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For patients with high RNP antibodies: › Seldom develop glomerulonephritis, cerebritis, psychosis, or seizures › Nearly always have development of Raynaud phenomenon › More likely to develop pulmonary hypertension › More likely to be RF positive (in 70%) and develop erosive arthritis
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4 features that suggest MCTD rather than another disorder: › Raynaud and swollen hands › Absence of renal and CNS disease › More severe arthritis › PHTN › RNP antibodies More common in women (16:1) Present in 2 nd or 3 rd decade
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Skin manifestations › Discoid plaques and malar rash Fever of unknown origin Arthritis is severe
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Myositis and myalgia Cardiac disease › RVH › Right atrial enlargement › Conduction defects › Pericarditis (10 to 30%)
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Pulmonary involvement (75%) › Effusions › PHTN Screen for with echo › Interstitial lung disease (30 to 50%) See septal thickening, ground-glass opacities, nonseptal linear opacities, and lower lobe predominance on CT scan › Infections › Vasculitis
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Absence of severe renal disease › High titers of RNP antibodies may be protective GI involvement in 60 to 80% › Hypomotility › Serositis › Mesenteric vasculitis › Pancreatitis CNS › Trigeminal neuropathy › Headaches
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Low grade anemia Leukopenia Hypergammaglobulinemia RF positive Anti-cyclic citrullinated peptide (CCP) antibodies + ANA RNP antibodies
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Thought to be incurable Some features are responsive to glucocorticoids Antimalrials or methotrexate should be used in patients requiring repeated or ongoing steroids Treat specific features (NSAIDS for arthritis, etc.)
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Mortality is 16 to 18% at 10 to 12 years Major cause of death is progressive pulmonary hypertension
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