Name
HENOCH-SCHONLEIN PURPURA
DESCRIPTION
DETAIL
DIFFERENTIAL DIAGNOSIS β’ Vasculitis β’ Allergic angiitis β’ Polyarteritis nodosum β’ Erythema multiforme β’ Inflammatory bowel disease (Crohn disease andulcerative colitis) β’ Systemic lupus erythematosus β’ Syphilis β’ Multisystem disease β’ Thrombophlebitis related to coagulation factor deficiency β’ Acute abdomen β’ Coagulopathies β’ Testicular torsion β’ Thrombocytopenic purpura β’ Sepsis β’ Acute glomerulonephritis β’ Acute hemorrhagic edema of infancy -------------------------------------------------------------------------- CAUSES β’ Probably an IgA-mediated small vessel vasculitis β’ Preceding streptococcal infection β’ Salmonella β’ Shigella β’ Yersinia β’ Legionella β’ Parvovirus β’ Adenovirus β’ Mycoplasma β’ Epstein-Barr virus β’ Varicella β’ Vaccination against typhoid, paratyphoid A and B, yellow fever and cholera β’ Toxocara canis β’ Penicillin, ampicillin β’ Erythromycin β’ Tetracycline β’ Vancomycin β’ Quinidine and quinine β’ Lisinopril β’ Enalapril β’ Losartan β’ Diphenhydramine β’ Tetramethylthiuram disulfide β’ Insect bitesOTHER TESTS : * URINE - PROTEINURIA & HAEMATURIA & RBC CAST. * BLOOD TEST - MILD LEUKOCYTOSIS, PLATELET COUNT - NORMAL, EOSINOPHELIA - OCCASIONALLY * IGA LEVELS - ELEVATED IN 50% β’ ESR elevated in 75% β’ ASO titer elevated 30% β’ CH50 - low in 30-40% β’ Properdin depression -30% β’ U/A: cellular casts indicate significant renal disease β’ Prothrombin deficiency β’ IgA immune complexes β’ Vitamin K deficiency β’ Factor VIII deficiency β’ Amylase, if pancreatitis suspected β’ Stool guaiac positive in half β’ IgD elevation β’ IgA1 O-linked oligosaccharide abnormalities, diminished galactose content β’ IgA anti-neutrophil antibody SPECIAL TESTS . Immunofl uorescent examination of involved tissue reveals IgA deposition . Anti-cardiolipin antibodies should probably be sought . Ultrasound - for intussusception . Pulmonary function tests - reduced diffusing capacity, but unclear that it is clinically significant IMAGING . X-ray . Uniform small bowel wall fold thickening - may even produce coin stack pattern . Edema and thumbprinting or filling defects of bowel walls . Intussusception DIAGNOSTIC PROCEDURES . Careful history and physical and frequent reevaluation
TYPENOTES
RISK FACTORS : Hypertriglyceridemia, HyperuricemiaAPPROPRIATE HEALTH CARE Supportive Measures GENERAL MEASURES o Sudden increase in abdominal pain may be caused by bowel infarction or perforation, gall bladder hydrops, intussusception or pancreatitis o Treatment of the underlying/associated infection o Discontinuation of unnecessary drugs o Closely monitor blood pressure, urinalysis and renal function o Leucocytopheresis DRUG(S) OF CHOICE o Nonsteroidal anti-inflammatory drugs at intermediate doses o Avoid prednisone for rash or abdominal pain, as aggravates systemic aspects of the disease o Prednisone - 1-2.5 mg/kg for renal disease (not just hematuria) or possible pulmonary hemorrhage o Azathioprine (Imuran) - 2 mg/kg/day ALTERNATIVE DRUGS o Full anticoagulation (PTT 60-80 seconds; PT INR 2.5-3.5) o Antiplatelet drugs - e.g., dipyridamole - 3 mg/kg to 6 mg/kg o The above two in combination with prednisone and cyclophosphamide o High dose immune globulin IV - 1 gm/kg/day in 12 hour infusions o Cyclosporine - 5 mg/kg/day o Cyclophosphamide - 2 mg/day in adults o Danazol 400-600 mg/day o Plasmapheresis o Recombinant Factor VIII infusion PATIENT MONITORING o Dependent on severity of system involvement o Long-term followup required if renal involvement, as progression may not be noted for years POSSIBLE COMPLICATIONS o Death o Renal failure o Bowel infarction or perforation o Gall bladder hydrops o Pancreatitis o Stenosing ureteritis o Isolated reports as presenting sign of cancer o Recurrence in renal transplant - 75% in living related donors; 0 in cadaver donors o Antiphospholipid syndrome EXPECTED COURSE/PROGNOSIS o Typically lasts 4-6 weeks o 50% have recurrences o Significant proteinuria, altered renal function, hypertension are adverse prognostic indicators o Rarely fatal
RELATED DISEASE
Not Available Disease
DISEASE
INVESTIGATION
ABSOLUTE EOSINOPHIL COUNT, BLEEDING TIME, CLOTTING TIME, SERUM IGA, URINE ROUTINE, ULTRA SOUND K.U.B., COMPLETE BLOOD COUNT