Findings SLE DILE CLINICAL Average age of onset of 2030 y Affects blacks more than whites Femaletomale ratio of 91 Average age of onset of 5070 y Affects whites more than blacks Femaletomale ratio of 11 ID: 1036829
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1. Drug Induced Lupus
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4. DILE following MINOCYCLINE for UTI
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6. FindingsSLEDILECLINICAL Average age of onset of 20-30 y Affects blacks more than whites Female-to-male ratio of 9:1Average age of onset of 50-70 y Affects whites more than blacksFemale-to-male ratio of 1:1LABORATORYAntihistone antibodies in 50%Anti-dsDNA present in 30-70% C3/C4 levels decrease Cutaneous findings in ~90% Raynaud phenomenon in 50% Antinuclear antibodies in >95%Antihistone antibodies in >95% Anti-ss DNA present ,Anti-ds DNA rareC3/C4 levels normal Cutaneous findings in ~25% Raynaud phenomenon in 25% Antinuclear antibodies in >95%
7. Mixed Connective Tissue Disease
8. The autoantibody pattern
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11. Gastrointestinal (GI) manifestations of MCTDSimilar to those of scleroderma:Upper and lower esophageal sphincter hypotension with gastroesophageal reflux leading to heartburn/dyspepsia (60%–70%)Esophageal dysmotility, and/or stricture with dysphagia (40%)Small bowel and colonic disease is less common in MCTD than in sclerodermaOther less common GI complications include Intestinal vasculitisAcute pancreatitisChronic active hepatitis
12. CNS manifestations of MCTDSevere CNS involvement is unusual Trigeminal neuralgia (most commonly) Sensorineural hearing loss Seizure and psychosis (rare)
13. Pulmonary manifestations of MCTDInvolvement of the lungs is common (75%) in MCTD, although most patients are asymptomaticAspiration secondary to esophageal disease contribute to pulmonary diseaseUp to 25% will develop severe pulmonary fibrosisThe most feared complication is PAH( major cause of mortality and morbidity )
14. Management Active inflammation, such as pleuritis, may respond to nonsteroidal antiinflammatory drugs (NSAIDs) or corticosteroidsOther medications such as AZA, MMF, or CYC may be used to treat ILD
15. Typical laboratory findings in a patient with MCTDAnemia of chronic diseaseCoombs’ positivity (in up to 60% of patients, although overt hemolytic anemia is uncommon)Thrombocytopenia is uncommonElevated ESR due to hypergammaglobulinemiaHypocomplementemia is not associated with any particular clinical manifestationAntibodies against common nuclear antigens (Rare)
16. U1-RNPU1-RNP is a uridine-rich small nuclear ribonucleoprotein (sn RNP) that consists of U1-RNA and U1-specific polypeptides 70kD, A& CPatients with MCTD form high titers of antibodies against U1-RNP, particularly U1-70kD and U1-RNA, but also polypeptides A& C, producing a high-titer ANA with a speckled patternAnti-U1-RNP antibodies can be present in other autoimmune diseases such as SLE and SSc, but typically in lower titer
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