Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + Download Section PDF Listen ++ For further information, see CMDT Part 20-13: Mixed Connective Tissue Disease & Overlap Syndromes + Key Features Download Section PDF Listen +++ ++ Many patients with symptoms and signs of a connective tissue disease have features of more than one type of rheumatic disease Special attention has been drawn to a subset of antinuclear antibody–positive patients who have high titers of RNP autoantibodies and overlapping features of SLE, scleroderma, rheumatoid arthritis and inflammatory myositis + Clinical Findings Download Section PDF Listen +++ ++ Swollen or puffy hands are a common early feature of disease Raynaud phenomenon, arthralgias and myalgias are common Unlike patients with SLE, renal or central nervous system disease is uncommon A key reason to identify this subset of patients is that pulmonary hypertension and interstitial lung disease are major causes of mortality, and regular screening for these manifestations is required + Diagnosis Download Section PDF Listen +++ ++ Clinical Table 20–7 Differential diagnosis SLE Scleroderma Polymyositis Sjögren syndrome Rheumatoid arthritis Eosinophilic fasciitis Graft-versus-host disease ++Table Graphic Jump LocationTable 20–7.Frequency (%) of autoantibodies in rheumatic diseases.1View Table||Download (.pdf) Table 20–7. Frequency (%) of autoantibodies in rheumatic diseases.1 ANA Anti-Native DNA Rheumatoid Factor Anti-Sm Anti-SS-A Anti-SS-B Anti-SCL-70 Anti-Centromere Anti-Jo-1 ANCA Rheumatoid arthritis 30–60 0–5 70 0 0–5 0–2 0 0 0 0 Systemic lupus erythematosus 95–100 60 20 10–25 15–20 5–20 0 0 0 0–1 Sjögren syndrome 95 0 75 0 65 65 0 0 0 0 Diffuse scleroderma > 95 0 30 0 0 0 33 1 0 0 Limited scleroderma (CREST syndrome) > 95 0 30 0 0 0 20 50 0 0 Polymyositis/dermatomyositis 80 0 33 0 0 0 0 0 20–30 0 Granulomatosis with polyangiitis 0–15 0 50 0 0 0 0 0 0 93–961 1Frequency for generalized, active disease.ANA, antinuclear antibodies; Anti-Sm, anti-Smith antibody; anti-SCL-70, anti-scleroderma antibody; ANCA, antineutrophil cytoplasmic antibody; CREST, calcinosis cutis, Raynaud phenomenon, esophageal motility disorder, sclerodactyly, and telangiectasia. + Treatment Download Section PDF Listen +++ ++ Guided more by the distribution and severity of organ system involvement than by therapies specific to these overlap syndromes