Henoch Schonlein Purpura (PowerPoint)

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Not Just a Pediatric Diagnosis

            Michael Craig
       Medicine Morning Report
          January 30, 2009
   Henoch-Schönlein purpura (HSP) is the most
    common systemic vasculititis in children, but
    can also be seen in adults
   In adults, tends to be more severe and have
    some differences in presentation
   20/100,000 children <17
   Peak is age 4-6 (70/100,000)
   In adults, estimated as 1/100 as frequent

   ~50% of childhood cases preceded by URI
    and/or strep pharyngitits
   In adults, malignancy thought to be frequent
     Adults HSP and Malignancy
   In retrospective review of 129 adult HSP
    patients with aged-matched controls
       relative risk of 5.25 with age-matched controls
       63% solid tumors, 37% hematologic
       more likely if older or male
       more frequent joint involvement
       more likely if no preceding infection
           Clinical Manifestations
   Tetrad of :
       Palpable purpura
       Arthritis/arthralgias
       Abdominal pain
       Renal disease
            Skin Manifestations
   Initially erythematous, macular or urticarial in
   Coalesces to ecchymoses, petechiae and
    palpable purpura
   Appears in symmetric crops
   Gravity-dependent (lower extremities)
   Usually (but not always) initial sign
           Joint Manifestations
   Transient or migratory
   1-4 joints
   Usually arthalgias without frank arthritis
   No long-term sequlae
                GI Manifestations
   Caused by submucosal hemorrhage and edema
   Range of severity of manifestations
       nausea, vomiting and abdominal pain
       GI bleeding, ischemia/necrosis, intussusception,
        bowel perforation
       rarely can see pancreatitis, gall bladder issues or
        bowel perforation
              Renal Manifestations
   Most with only mild involvement
       Asymptomatic hematuria, proteinuria, mild creatinine
   Some may be more severe
       Nephrotic syndrome, hypertension, acute renal failure
   Severity tends to correlate with biopsy findings
       mild mesangial proliferation vs. cellular proliferation vs.
        crescent formation
       Immunofluorescence shows mesangial IgA deposition +/-
        IgG, IgM or C3 deposits
             Differences in Adults
   Skin and joint symptoms are more often the
    presenting feature
   GI symptoms are less common
       Intussuception is rare
   Renal involvement more common and tends to
    be more severe
       ESRD in 10% vs. 0% in one retrospective review
   In children, usually a clinical diagnosis
       biopsies reserved for unusual presentations
   Skin biopsy
       demonstrates leukocytoclastic vasculitits with significant
        IgA deposition
       biopsy lesions less than 24 hours old if possible
   Kidney biopsy
       if severe renal involvement or diagnostic uncertainty
          Other Diagnostic Studies
   All patients should get U/A
       All adults (or children with abnormal U/A) should get
        creatinine checked
   Platelet count and coagulation studies to evaluate for
    other causes of purpura
   May be elevated markers of inflammation if triggered
    by bacterial infection, although may be normal in
    viral infection
   If concerned about severe GI manifestations,
    abdominal imaging
       Ultrasound study of choice for intussception
       CT may be more useful in adults
              Differential Diagnosis
   Hypersensitivity vasculitits
   Other small vessel vasculitides (Wegener’s, microscopic
    polyangitis, Churg-Strauss, SLE, secondary vasculitis, etc.)
       Skin biopsy with IgA deposition conclusive
       Other labs (ANCA, ANA, complement, etc.) can be helpful in
   Other causes of purpuric/petechial rashes (sepsis, ITP,
    coagulopathy, etc.)
   SLE, JIA, rheumatoid arthritis (joint pain)
   Surgical abdomen (abdominal pain)
   IgA nephropathy (renal involvement)
                Diagnostic Criteria
   American College of Rheumatology classification
       Palpable purpura
       Age of onset <= 20 years
       Acute abdominal pain
       Biopsy showing granulocytes in walls of small arterioles
        and/or venules
   2 or more critieria estimated ~90% sensitive and
    specific in adults for distinguishing HSP from other
   Supportive care
   NSAIDs for joint / abdominal pain
       Not thought to increase risk of GI bleeding
   Steroids?
       May decrease duration of abdominal pain
       Not been shown to decrease severity of renal disease
       Beware disease flare-up when tapering
   Other immunosuppressives for renal disease?
       Azathioprine, cyclophosphamide, plasmapheresis have been used, but
        no good controlled trial data
   Transplant for ESRD?
       35% recurrence at 5 years, 10% lose graft due to recurrence
            Take-Home Points
   HSP is less common but possible in adults
   Always biopsy skin lesions if suspected
   In adults diagnosed with HSP, consider
    malignancy as a possible trigger
   In adults, HSP tends to have more joint
    symptoms, more severe renal disease and less
    abdominal involvement
   UpToDate
   Harrison’s Principles of Internal Medicine
   Panhurst T, Savage COS, Gordon C and Harper L. Malignancy is increased
    in ANCA-associated vasculitits. Rhematology 2004;43:1532-5.
   Kellerman PS. Henoch-Schonlein Purpura in Adults. Am J Kidney Dis.
   Mills JA et. Al. The American College of Rheumatology 1990 criteria for
    the classification of Henoch-Schonlein Purpura. Arthritis Rheum.

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