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1 ã Vasculitis CONCEPT ○ Tearing of RBC and platelets ○ Physical Manifestations  Petechiae  Purpura  Ecchymoses = large bruise ○ When a blood vessel tears → CLOT FORMATION → ↓ radius of the vessel, therefore ↑ R = ↑ BP ○ ↓ Flow to the kidney = Ischemia!!!! 7 patterns in the kidney of vasculitis: ã partial clot in renal artery  renal stenosis ã Complete clot inrenal artery  renal failure ã Inflammed glomerulus  glomerular nephritis ã Clot off medulla  interstisial nephritis ã Clot off Pap
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  ã Vasculitis CONCEPT ○ Tearing of RBC and platelets ○ Physical Manifestations  Petechiae  Purpura  Ecchymoses = large bruise ○ When a blood vessel tears CLOT FORMATION radius of the vessel, →→↓  therefore R = BP ↑↑ ○ Flow to the kidney = Ischemia!!!! ↓ 7 patterns in the kidney of vasculitis: ã partial clot in renal artery  renal stenosis ã Complete clot inrenal artery  renal failure ã Inflammed glomerulus  glomerular nephritis ã Clot off medulla  interstisial nephritis ã Clot off Papilla  . papillary necrosis ã Clott of pieces of nephron  focal segmental glomerulo nephritis ã Clot off all the nephrons  RPGN = rapid progressive glomerulo nephritis The two patterns that goes with everything else: ã Membranous  If the disease involves deposition of anything ○ Will always be the first manifistation of disease. ã Membranoprolifirative  deposition of anything and also autoimmune complexdeposition. ○ Ex. That’s why Lupus is the only disease/vasculitis that will die from renal disease andnot HF, because patient can get every possible renal complication. ○ Any chronic disease/drug use can cause depositions!!!1  Berger’sIgA deposition 2 weeks after URIMC Nephritic syndrome in Children Alports Deafness, cataracts run in the family IgA nephropathyHenoch-Schonlein PurpuraIgA nephropathy 2 weeks after diarrheaPetechiae start from the hips/butt down 2 Complication ã GI bleeding ã Intussusception – current jelly stool(blood+stool), sausage shaped mass ONLY vasculitis with NORMAL plateletcount Buergers Smoking Jewish personnecrotizing vasculitis DICD-dimersFibrin split productsMost common cause of sepsis (endotoxin)HUS2 weeks after diarrhea ( E.coli/raw hamburger ) MCC of renal failure in Children TTP –thrombotic cytopenic purpura Neurological changes Triad:1.Fever2.Thrombocytopenia3.Neurological changes Polyarteritis Nodosa pANCA Hepatitis B association in 40% of casesAffects medium sized arteries (GI/Renal)Wegner’s cANCA involves the sinuses, lungs and kidneys at thesame time#2 cause of RPGNPost strep GN 2 weeks after sore throat Low complement Strain 12DM Glove and stocking neuropathy Does not have immune complexes therefore, canonly progress to Focal Segmental GNCREST =mild form of scleroderma C alcinosis R aynaud’s (spontaneous vasospasms) E sophogeal dismotility (due to scarring)   2  S clerodactyly (tightning of fingers/toes) T elangectasiaClue: anti centromere antibodyMild form of SclerodermaSclerodermaTightning of skin and smooth muscleAnti-smooth muscle antibodyAnti – Scl70Progressive Systemic SclerosisOrgans are scarred upAnti - topoisomeraseMixed Connective Tissue Disease, MCTDcombinationAnti- ribonuclar proteinGoodpasturesLungs and kidney Anti-GBM#1 rapidly progressive diseaseLinear immunofluoresence SLEAnti-ds DNA, Anti-smith, Anti-cardiolipinDie from renal failureSubepithelial humpsSerum sickness2 weeks after a vaccine (especially MMR)Can be due to drugsSBE-subachute endocarditisseptic emboli in the brain – micotic aneurysmroth spots – to retinaoslors nodes – to fingerssplinter hemorrhages – nail bed janeway lesions - toesBacteria: Streptococcus ViridansMitral valve involvement (prolapse)MPGNType I C → 3 and nephritic factor in BMType II complement; dense deposit disease →↓ Both have a tram track appearanceCryoglobulinemiaIgMAcute inflammation onlyNon-bacterialInfluenzaAdenovirusMycoplasmaHep B3  EBVRAanti- IgGMost common arthritis in middle age females1.symmetrical2.polyarthritis (many joints affected)3.Involves synovium/Pannus4.Worse in A.M.5.Periosteal erosions on side of the bone.JRAStills Disease2/3 are Rheumatoid Factor negative – goodprognosisIridocyclitis – inflammation of iris and circularmuscles of the eye.  very bad complicationNeed to see opthamologist every year.1/3 who are RF positiverampant diseasepoor prognosisAffects Pannus ( sinovial lining)FeltysRA + leucopenia + splenomegalyBecetsRA + GI ulcersSjogrensRA+xeropthlmia/xerostomiaAnti- Ro, la, ssaincidence of heart block in newborns ↑ SiccaWithout RASyphillisObliterative endarteritis d/t gumma depositionLeukocytoclasticDrug allerf=gyChurg StrausNecrotizing vasculitis d/t parasites in the lungsSee eosinophils ↑ IdiopathicTakayasuPulseless Japanese WomanKawasakiOnly one with a HIGH platelet countJapanese childrenStrawberry tongueRash on the PALMS & SOLES!!!HTNMCC of Vasculitis in adults!!!4
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