Adult onset HenchSchonlein Purpura A case report

1
Adult onset Henöch-Schonlein Purpura A case
report

• Dr.Tamilselvam Tiruchengode
• Department of Rheumatology
• Madras Medical College
• Chennai, India.

2
History

• Presenting complaints
• 40 year old male-
• Admitted with
• Erythematous raised skin rash 3 months
• Involving both upper and lower limbs, lower
  abdomen and gluteal region
• Non pruritic

3
History

• Pain and swelling of both elbows and knees 1
  month (no other joints involved)
• Abdominal pain 1 month
• Vague dull aching, diffuse
• Intermittent unrelated to food
• Symptoms progressed initially for 3 months and
  remained static thereafter
• No constitutional features

4
History

• No h/o any recent medications intake
• No h/o any recent upper respiratory tract or
  other infections
• No h/o oral / nasal ulcers, alopecia,
  photosensitivity, Raynauds
• No h/o myalgia, weakness or weight loss
• No h/o wheeze, hemoptysis

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Investigations

• Hb 9.7 g/dl
• WBC 4200 cells/mm3
• Differential count
• P 53,L 44,E 3
• ESR 25 mm / hr
• CRP - lt1 mg/L
• Platelets- 175,000/cmm

• Creatinine 0.7 mg/ dl
• AST 40 IU/ dl
• ALT 35 IU/ dl
• Amylase 152 IU / dl
• Cholesterol 210 mg/ dl
• Triglycerides 130 mg/ dl
• LDL 140 mg/ dl
• HDL 40 mg/ dl

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Investigations Antibodies

• RF (latex) Negative
• ASO Negative
• ANA Negative
• (Hep 2)
• HBsAg Negative
• ANCA- Negative

• Anti HCV Negative
• Cryoglobulin Negative
• Cryofibrinogen Negative
• Serum Ig A 556 mg / dL
• (Normal 68 378 mg/dl)

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Investigations - Imaging

• Abdominal ultrasound Normal study
• Chest X ray Normal
• X ray both elbows and knees Normal
• CT abdomen Normal study
• UGI Endoscopy - Normal

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Skin biopsy

• Light microscopy leukocytoclastic vasculitis
• Immunofluorescence
• -Ig G Negative
• -IgM Negative
• -Ig A granular fluorescence in the papillary
  dermal vessels
• -C 1 q Negative
• -Fibrinogen Negative

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Case Summary

• 40 yrs male
• Palpable purpura involving dependent areas of
  trunk and extremities with normal platelet counts
• Non-specific abdominal pain
• Predominantly lower limb oligoarthritis
• Absence of constitutional features
• No precipitating or preceding illness
• Elevated serum IgA levels
• Skin biopsy with leukocytoclastic vasculitis and
  positive for IgA and C3 immune deposits by IF

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Differential Diagnosis

• Henöch-Schonlein purpura
• Hypersensitivity vasculitis
• Classic PAN, Cutaneous PAN
• Essential Mixed Cryoglobulinemia
• Isolated Cutaneous Leukocytoclastic Vasculitis
• Paraneoplastic Vasculitis
• Wegeners, Churg-Strauss
• Vasculitis secondary to systemic autoimmune
  disease (SLE,RA,SS)
• HCV associated cryoglobulinaemia
• Immune complex disorders (IBD, Sarcoid,CF)

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HSP in Adults

• Age range 34 - 53 years
• Palpable purpura with a normal platelet count
  (100)
• Arthritis (large joints MCP,PIP,MTP)
• Benign nature of all features except renal
• More skin necrosis in adults
• High serum IgA levels Favorable clinical course
• Adults - melena, hematuria, renal insufficiency
• IgA immune deposits in skin or renal biopsy

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ACR CRITERIA

• Age lt 20 years or younger
• Palpable purpura
• Bowel angina
• Typical biopsy findings
• Two or more criteria have a sensitivity and
  specificity of approximately 87

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NEW EULAR CRITERIA 2006

• Deleted Age at onset
• Inclusion Predominant IgA deposition Arthritis
  Renal involvement
• Suggestions
• GI involvement more appropriate than diffuse
  abdominal pain or bowel angina.

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Renal manifestations

• Abnormalities of urinary sediment
• Acute nephritis
• Progressive renal disease
• Glomerular crescent formation - fatal outcome
• Mortality 4
• Purpura above wrist, pyrexia, elevated acute
  phase reactants, antecedent upper respiratory
  infection- IgA glomerulonephritis
• Nephropathy adults 85 children 25
• Renal insufficiency 13 in adults, 0 in children

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Gastrointestinal manifestations

• One third with HSP have GI features
• Colicky pain, nausea, vomiting, GI bleed
• 50 have occult bleeding
• Major GI bleed in 5
• Intussusception 2
• Abdominal pain sub-mucosal and intramural
  extravasation of fluid and blood
• Endoscopy Erosive duodenitis

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Genitourinary involvement

• Extra renal genitourinary involvement may precede
  rash
• Acute scrotal swelling due to involvement of
  scrotal vessels
• Testicular torsion vs HSP scrotal pain
• Role of Doppler flow and radionuclide studies to
  avoid unwarranted surgery
• Testicular torsion - surgical emergency

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HSP in Children Adults
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Pathogenesis of HSP

• Immune complex mediated small vessel vasculitis
• Increased serum IgA in 50
• Complement activation, chemotaxis,
• recruitment of neutrophils, lysosomal enzyme
  release and vessel injury
• Membrane Attack Complex - mediated damage

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Pathogenesis - New Concepts with Potential for
New Biomarkers

• HLA B 35 DR4-Nephritis
• C4 null allele
• IL-1RA allele 2 - Nephritis
• IL-1,IL-6- active disease
• Endothelins-TNF alpha
• vWf
• IgA
• IgA ANCA
• IgA Antibodies - HUVEC
• Anti-endothelial cell antibodies
• Nitric oxide
• D-Dimer

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Poor prognostic factors

• Nephrotic syndrome, renal insufficiency
• Renal failure, hematuria, nephrotic range
  proteinuria
• 15 risk of renal failure
• Indication for renal biopsy
• Nephrotic range proteinuria
• Nephrotic syndrome
• Renal failure

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Management of HSP

• Supportive therapy - organ and system oriented
  approach
• NSAIDS
• Help reducing joint pain without aggravating
  purpura
• Steroids
• Acute hemorrhagic edema
• Reduction of abdominal pain in the first 24-48
  hrs
• Reduction melena, massive hemorrhage
• Prevention of delayed nephritis
• Necessity for long-term follow up to anticipate
  renal dysfunction

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Outcome of Patient

• Treated with Prednisolone 25mg for 2 weeks and
  tapered off over 3 months
• Skin lesions, abdominal pain and arthritis
  resolved after 1 month
• No recurrence normal renal parameters
• Followed for 19 months
• Under follow up and regular review