1 / 15

Good Morning ! October 3 rd , 2011

Good Morning ! October 3 rd , 2011. Mixed Connective Tissue Disease. An overlap syndrome associated with anti-U1-RNP (ribonucleic protein) antibodies with features of SLE, scleroderma, and polymyositis

charis
Download Presentation

Good Morning ! October 3 rd , 2011

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. Good Morning ! October 3rd, 2011

  2. Mixed Connective Tissue Disease • 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)

  3. Is MCTD a specific disease? • Early symptoms • Easy fatigability • Poorly defined myalgias • Arthralgias • Raynaud phenomenon

  4. Major reason to consider MCTD a distinct entitiy • 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

  5. Clinical features • 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 2nd or 3rd decade

  6. Clinical features (cont.) • Skin manifestations • Discoid plaques and malar rash • Fever of unknown origin • Arthritis is severe

  7. Clinical features (cont.) • Myositis and myalgia • Cardiac disease • RVH • Right atrial enlargement • Conduction defects • Pericarditis (10 to 30%)

  8. Clinical features (cont.) • 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

  9. Clinical features (cont.) • 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

  10. Lab abnormalities • Low grade anemia • Leukopenia • Hypergammaglobulinemia • RF positive • Anti-cyclic citrullinated peptide (CCP) antibodies • + ANA • RNP antibodies

  11. Treatment • 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.)

  12. Prognosis • Mortality is 16 to 18% at 10 to 12 years • Major cause of death is progressive pulmonary hypertension

  13. Noon Conference Radiology

More Related