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Mixed connective tissue disease
- 1. Mixed Connective Tissue Disease SAMIREL ANSARY ICU PROFESSOR AIN SHAMS CAIRO
- 2. Global Critical Care https://www.facebook.com/groups/1451610115129555/#!/groups/145161011512 9555/ Wellcomein our new group ..... Dr.SAMIR EL ANSARY
- 3. “Mixed Connective Tissue Disease: StillCrazy After All These Years”
- 4. History • 1972 Sharpand colleagues – Identified patients with high levels of antibodies against a ribonucleic protein (RNP) – These patients shared several clinical features including Raynaud’s phenomenom, arthralgias, mild arthritis, puffy hands, abnormal esophageal mobility, and myositis – Additional findings – hypergammaglobulinemia (80%), anemia and leukopenia (50%); pulmonary, renal and CNS involvement was “rare”
- 5. History • 1980 Nimelsteinand colleagues – Doubts after reviewing 22/25 original patients – Many patients evolved into scleroderma – High mortality rate (8/22) – Not everyone had antibodies to RNP – Some patients had antibodies without clear clinical features of the syndrome • Then, 20 years of contradictory views regarding the existence and nature of MCTD
- 6. Diagnostic Criteria • Featuresof SLE, systemic sclerosis, RA, and polymyositis • Four different diagnostic criteria have been proposed – Sharp – Kasukawa – Alarcon-Segovia – Kahn • Highest sensitivity (62%) and specificity (86%) with Alarcon-Segovia and Kahn in 45 patients
- 7. Diagnostic Criteria –Sharp • Major Criteria – Myositis – Pulmonary Involvement – Raynaud phenomenom or esophageal dysmobility – Swollen hands or sclerodactyly – High anti-U1-RNP with negative anti-Sm • Definite – 4 major plus serology • Probable – 3 major or 2 major (1st 3 listed) and 2 minor; and serology • Minor Criteria – Alopecia – Leukopenia – Anemia – Pleuritis – Pericarditis – Arthritis – Trigeminal Neuralgia – Malar Rash – Thrombocytopenia – Mild Myositis – h/o swollen hands
- 8. Diagnostic Criteria Alarcon-Segovia –Clinical Criteria3/5 (must have synovitis or myositis) • Edema of the hands • Synovitis • Myositis • Raynaud’s phenomenon • Acrosclerosis Serologic: high titers of anti-U1 RNP
- 9. Diagnostic Criteria • “Thecrux of the MCTD diagnosis is the presence of high titers of antibodies to U1-RNP.” • Many patients who satisfy criteria for MCTD also satisfy ACR criteria for RA or SLE, and many had symptoms of systemic sclerosis. • “With serology superseding the clinical symptoms in the diagnosis, there is a risk of fitting the clinical symptoms to the antibody signs”
- 10. Clinical Presentation • EarlyClinical Findings – Malaise, easy fatiguability – Arthralgias – Myalgias – Raynaud’s phenomenom – Low-grade fevers • Unusual Presentations – FUO Fever of unknown origin – Serositis – Trigeminal neuropathy – Severe polymyositis – Acute arthritis – Aseptic meningitis – Digital gangrene
- 11. Characteristic At DiagnosisCumulative at 5 years Raynaud’s Phenomenom 89% 96% Arthralgia/Arthritis 85% 96% Swollen Hands 60% 66% Esophageal Dysmotility 47% 66% Pulmonary Dysfunction 43% 66% Serositis 34% 43% Hematologic 30% 53% Erythematous Skin Rash 30% 53% Muscle Myositis 28% 51% Pulmonary Hypertension 9% 23% Sclerodermatous Changes 4% 19% CNS (or peripheral) 0% 17% Renal 2% 11%
- 12. Pulmonary Manifestations • PleuralEffusions • Pulmonary Hypertension • Pleuritic Pain • Intersitial Lung Disease (30-50%) • Thromboembolic Disease • Obstructive Disease • Pulmonary Vasculitis • 75% of patients • Early Symptoms – Dry cough – Dyspnea – Pleuritic Chest Pain
- 13. Pericardial Disease • PericardialInvolvement – Scleroderma 59% – SLE 44% – MCTD 30% – RA 24% • MCTD – At autopsy – 56% had pericardial disease – Asymptomatic pericardial effusion – 24-38%
- 14. Laboratory Findings • Hightiter, speckled ANA pattern • Leukopenia, anemia, thrombocytopenia • Elevated ESR • Very high serum immunoglobulins • Complement levels usually normal or high • Rheumatoid Factors increased in 70% of patients • Negative findings include anti-dsDNA and anti-Sm antibodies (if positive, some argue that it represents exclusion criteria for MCTD)
- 15. Antibody Findings Disease ANARF dsDNA Sm Scl-70 RNP SLE 95-99 20 50-70 30 0 30-50 RA 15-35 85 <5 0 0 10 Diffuse SSc >90 30 0 0 40 30 MCTD 95-99 50 0 <5 0 100
- 16. Follow-Up • 39 MCTDpatients at 10 year follow-up – 64% “differentiated” into another syndrome – 11 systemic sclerosis, 10 SLE, 2 RA, 2 overlap syndrome • Other studies have found similar results • About 40% of patients with anti-U1RNP antibodies retain the diagnosis of MCTD and others are “reclassified” within 5 years of presentation
- 17. Undifferentiated and Overlap Syndromes •MCTD –SLE, SSc, PM, RA • Undifferentiated Systemic Rheumatic Disease –Undifferentiated connective tissue, collagen, vascular, or autoimmune disease • Nonclassical SLE • “Atypical” rheumatic disease • Undiiferentiated Polyarthritis Syndrome • Undifferentiated Spondyloarthritis
- 18. Undifferentiated and Overlap Syndromes OverlapSyndromes • RA-lupus – Rhupus • Scleroderma-PM/DM • Scleroderma-lupus • Scleroderma-PBC-Sjogren’s • Scleroderma-RA JRA-lupus Psoriatic arthritis-lupus Psupus Sjogren’s overlaps PM overlaps Raynaud’s phenomenom overlaps
- 19. SLE Criteria • MalarRash • Discoid Rash • Photosensitivity • Oral Ulcers • Arthritis • Serositis • Renal Disease • Neurologic Disease • Hematologic Disease – Hemolytic anemia – Leukopenia, lymphopenia • Immunologic – Anti-dsDNA – Anti-Sm • ANA • 4/11 Criteria
- 20. Global Critical Care https://www.facebook.com/groups/1451610115129555/#!/groups/145161011512 9555/ Wellcomein our new group ..... Dr.SAMIR EL ANSARY
- 21. GOOD LUCK SAMIR ELANSARY ICU PROFESSOR AIN SHAMS CAIRO elansarysamir@yahoo.com