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Mixed Connective Tissure Disease

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Mixed Connective Tissure Disease

  1. 1. Grand Round <ul><li>主講者 : 李振威 </li></ul><ul><li>96-10-09 </li></ul>
  2. 2. Mixed Connective Tissue Disease <ul><li>1.The central premise of MCTD is that of an </li></ul><ul><li>overlap syndrome embracing features of </li></ul><ul><li>SLE. Scl and PM/DM. </li></ul><ul><li>2.Firstly described by Sharp and his collea- </li></ul><ul><li>-gues in a 1972 papper report. </li></ul><ul><li>3.This was the first overlap syndrome defin </li></ul><ul><li>ed in terms of a specific Ab. </li></ul>
  3. 3. Serological Features <ul><li>1.The basic premise of MCTD is that the pre </li></ul><ul><li>sence of high titer anti-U1 RNP. </li></ul><ul><li>2.The first clue to diagnosing MCTD is usua </li></ul><ul><li>lly a positive ANA with a high titer speckle </li></ul><ul><li>pattern. </li></ul><ul><li>3.The titer is often greater than 1:1000 and </li></ul><ul><li>sometimes greater than 1:10000 </li></ul>
  4. 6. <ul><li>4.The finding should prompt the measure </li></ul><ul><li>ment of Ab to U1 RNP,Sm,Ro and La. </li></ul><ul><li>5.Ab.to ds-DNA,Sm and Ro. Are occasion- </li></ul><ul><li>-ally seen as a transient phenomenon in </li></ul><ul><li>patient with MCTD. </li></ul><ul><li>6.68 Kd U1 RNP specific polypeptide was </li></ul><ul><li>identified as the disease-defining target </li></ul><ul><li>Ab. Of MCTD </li></ul>
  5. 7. Clinical Features <ul><li>1.The central premise of MCTD IS THAT </li></ul><ul><li>of an overlap syndrome embracing fea </li></ul><ul><li>-tures of SLE,Scl and PM/DM. </li></ul><ul><li>2.These overlap features seldom occur </li></ul><ul><li>concurrently. </li></ul><ul><li>3.The symptom of early phase are hand </li></ul><ul><li>edema,arthritis,Raynaud’s phenomenon </li></ul><ul><li>and inflammatory muscle disease. </li></ul>
  6. 8. Diagnostic Criteria <ul><li>1.Alarco’n-Segovia’s Criteria: </li></ul><ul><li>-A: serological criteria: </li></ul><ul><li>-Anti-RNP at hemagglutination </li></ul><ul><li>-B: clinical criteria </li></ul><ul><li>-swollen hand,synovitis,myositis </li></ul><ul><li>raynaud’s phenomenon.acrosclerosis </li></ul>
  7. 9. Kahn’s Criteria <ul><li>A :serological criteria </li></ul><ul><li>-high titer anti-RNP </li></ul><ul><li>B: clinical criteria </li></ul><ul><li>swollen fingers </li></ul><ul><li>synovitis </li></ul><ul><li>myositis </li></ul><ul><li>Raynaud’s phenomenon </li></ul>
  8. 16. General Features and Epidemiology <ul><li>1.Prevalence: 2.7% </li></ul><ul><li>2.female-to-male ratio:16:1 </li></ul><ul><li>3.age at onset:20-30 years old </li></ul>
  9. 17. Early Symptoms <ul><li>1.In the early stage, most pt to develop </li></ul><ul><li>MCTD cant be differentiated from the </li></ul><ul><li>other classic DCTD. </li></ul><ul><li>2.Early in the course of the disease most </li></ul><ul><li>pt. complain of easy fatigability,poorly </li></ul><ul><li>defined myalgia,arthralgia and Raynaud </li></ul><ul><li>phenomenon. </li></ul>
  10. 18. <ul><li>3.If such a pt. is found to have swollen </li></ul><ul><li>hands,puffy fingers,or both in association </li></ul><ul><li>with a high titer,speckle ANA, they shou- </li></ul><ul><li>-ld be carefully followed for the evolution </li></ul><ul><li>of overlap features. </li></ul><ul><li>4.A high-titer of anti-RNP Ab. In a pt with </li></ul><ul><li>UCTD is a powerful predictor for MCTD. </li></ul>
  11. 19. FEVER <ul><li>1.Fever may be a prominent feature of </li></ul><ul><li>MCTD in the absence of an obvious cau- </li></ul><ul><li>-se. </li></ul><ul><li>2.F.U.O has been the initial presentation </li></ul><ul><li>of MCTD. </li></ul><ul><li>3.Fever in MCTD can usually be traced to </li></ul><ul><li>a coexistent myositis,aseptic meningitis </li></ul><ul><li>serositis,lymphadenopathy,or intercurrent inf. </li></ul>
  12. 20. JOINTS <ul><li>1.Joint pain and stiffness is an early symp </li></ul><ul><li>tom in nearly all patients who develop </li></ul><ul><li>MCTD syndrome. </li></ul><ul><li>2.Joint involvement in MCTD is more com- </li></ul><ul><li>-mon and more severe than in classic SLE </li></ul><ul><li>3.About 60% pt eventually develop an </li></ul><ul><li>obvious arthritis. </li></ul>
  13. 21. <ul><li>4.Radiograph usually show a characteristic </li></ul><ul><li>absence of severe erosive change </li></ul><ul><li>5.Small marginal erosion,often with a </li></ul><ul><li>well-demarcated edge,are the most </li></ul><ul><li>characteristic radiological feature in pt </li></ul><ul><li>with severe joint disease. </li></ul>
  14. 22. <ul><li>6.A positive RF is found in 50-70% of pt </li></ul><ul><li>with MCTD,indeed,pt may be diagnosed </li></ul><ul><li>as having RA and fulfill ACR criteria for </li></ul><ul><li>RA. </li></ul><ul><li>7.Joint histology in MCTD reveal a hyperp </li></ul><ul><li>lastic synovium with surface fibrinoid ne </li></ul><ul><li>-crosis,increased vascularity,interstitial </li></ul><ul><li>edema and infiltration of macrophage,lympho </li></ul><ul><li>cyte,neutrophil and multinuclear giant cell. </li></ul>
  15. 23. Skin and Mucous Membranes <ul><li>1.Raynaud phenomenon is the most com- </li></ul><ul><li>-mon problem and one of the earliest </li></ul><ul><li>manifestation of MCTD. </li></ul><ul><li>2.In some pts ,skin change commonly ass </li></ul><ul><li>-ociated with SLE are prominent finding </li></ul><ul><li>particularly malar rash and discoid rash. </li></ul>
  16. 25. <ul><li>3.Mucous membrane lesions have included </li></ul><ul><li>buccal ulceration,livedo vasculitis,sicca </li></ul><ul><li>syndrome,oralgenital ulceration and </li></ul><ul><li>nasal septal perforation. </li></ul>
  17. 26. MUSCLE <ul><li>1.Sometimes an inflammatory myositis </li></ul><ul><li>is seen in patient with anti-U1RNP who </li></ul><ul><li>do not have the feature of MCTD. </li></ul><ul><li>2.The inflammatory myopathy associated </li></ul><ul><li>with MCTD is identical clinically and his- </li></ul><ul><li>-tologically to classical PM. </li></ul>
  18. 27. HEART <ul><li>1.All three layers of the heart may be </li></ul><ul><li>involved in MCTD. </li></ul><ul><li>2.20% of MCTD pt EKG abnormal. </li></ul><ul><li>3.The most common EKG findings are RVH </li></ul><ul><li>,RAE and interventricular conduction </li></ul><ul><li>defect. </li></ul>
  19. 28. <ul><li>4.Pericarditis is the most common clinical </li></ul><ul><li>finding of cardiac involvement. </li></ul><ul><li>5.Early detection of pul. Hypertension is </li></ul><ul><li>important for initiating early therapy. </li></ul>
  20. 29. LUNG <ul><li>1.Pleuropulmonary involvement in 25% </li></ul><ul><li>of patient. </li></ul><ul><li>2.Symptoms were dyspnea,chest pain and </li></ul><ul><li>cough. </li></ul><ul><li>3.CXR showed interstial change,pleural </li></ul><ul><li>effusion,pneumonic infiltration and pleu- </li></ul><ul><li>-ral thickening </li></ul>
  21. 30. <ul><li>4.The most discriminatory lung function </li></ul><ul><li>test is DLCO. </li></ul><ul><li>5.High-resolution CT is the most sensitive </li></ul><ul><li>test to determine the presence of inter- </li></ul><ul><li>-stitial lung disease in MCTD. </li></ul><ul><li>6.The major cause of death in MCTD is </li></ul><ul><li>pul. H/T </li></ul>
  22. 31. <ul><li>7.Intimal hyperplasia and smooth muscle </li></ul><ul><li>hypertrophy without accompanying </li></ul><ul><li>inflammation are the characteristic fea- </li></ul><ul><li>-ture of vasculopathy in MCTD. </li></ul>
  23. 32. KIDNEY <ul><li>1.Renal involvement occurred in 25% </li></ul><ul><li>MCTD pt. </li></ul><ul><li>2.High titer of anti-U1RNP Ab are relative </li></ul><ul><li>-ly protective against the development </li></ul><ul><li>diffuse proliferative GN. </li></ul><ul><li>3.When pt do develop renal change,they </li></ul><ul><li>usually develop membranous GN </li></ul>
  24. 33. GASTROINTESTINAL <ul><li>1.A major feature of MCTD, occurring in </li></ul><ul><li>60-80 % of pt. </li></ul><ul><li>2.The most common abdominal problem </li></ul><ul><li>in MCTD is disordered mortility in the </li></ul><ul><li>upper GI tract. </li></ul><ul><li>3.Abdominal pain in MCTD may result from bowel hypomotility,serositis, </li></ul>
  25. 34. <ul><li>Mesenteric vasculitis,colonic perforation </li></ul><ul><li>and pancreatitis. </li></ul><ul><li>4.Malabsorption syndrome can occur sec </li></ul><ul><li>ondary to small bowel dilatation and bac </li></ul><ul><li>teric overgrowth. </li></ul>
  26. 35. Central Nervous System <ul><li>1.There is general agreement that the </li></ul><ul><li>most common problem is a trigeminal </li></ul><ul><li>neuropathy. </li></ul><ul><li>2.Headache is a relative common symp </li></ul><ul><li>-tom in MCTD, in the majority of pt they </li></ul><ul><li>are probably vascular in origin with cla- </li></ul><ul><li>-ssical migrain. </li></ul>
  27. 36. Blood Vessel <ul><li>1.A bland ,intimal proliferation and medial </li></ul><ul><li>hypertrophy affecting medium and small </li></ul><ul><li>vessels is the characteristic vascular les- </li></ul><ul><li>-ion of MCTD. </li></ul><ul><li>2.When the condition is widespread in the </li></ul><ul><li>lung and kidney,there is association with </li></ul><ul><li>pul. H/T and renal crisis. </li></ul>
  28. 37. <ul><li>3.An angiographic study reported a high </li></ul><ul><li>prevelence of medium-size vessel occl- </li></ul><ul><li>-usion. </li></ul><ul><li>4.Fingernail capillaroscopy showed bushy </li></ul><ul><li>like lesion(capillary dilatation and drop out) </li></ul>
  29. 39. <ul><li>5.Anti-endothelial cell Ab. Have been rep- </li></ul><ul><li>-orted in 45% pt,the condition correlate </li></ul><ul><li>with pul. Change and spontaneous abor- </li></ul><ul><li>-tion. </li></ul><ul><li>6.Anti-U1 RNP AB. May have a pathologic </li></ul><ul><li>role in the small-vessel pathology </li></ul>
  30. 43. BLOOD <ul><li>1.Anemia of chronic disease is found in 75% </li></ul><ul><li>of pt. </li></ul><ul><li>2.RF. Positive in 50% of patient,the con- </li></ul><ul><li>-dition is associated with severe arthritis. </li></ul>
  31. 44. Prognosis <ul><li>1.Pt with high-titer U1RNP Ab. Have a low </li></ul><ul><li>prevalence of serious renal disease and </li></ul><ul><li>life-threatening neurological problem. </li></ul><ul><li>2.Pul. H/T sometime follow a rapid acce- </li></ul><ul><li>-lerated course, which lead death in a </li></ul><ul><li>few weeks. </li></ul>
  32. 45. <ul><li>3. 5 years survival rate was 90.5% </li></ul><ul><li>10 years survival rate was 82.1% </li></ul><ul><li>4.Pt with predominent Scl-PM overlap with </li></ul><ul><li>a worse prognosis. With a 33% 10 years </li></ul><ul><li>survival rate. </li></ul>
  33. 46. Management <ul><li>1.There is no cure for MCTD. </li></ul><ul><li>2.Pul. H/T is the main cause of death. </li></ul><ul><li>3.Recommendation for management are </li></ul><ul><li>base on conventional treatment for SLE </li></ul><ul><li>PM,DM,RA and Scl. </li></ul>

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