Hypersensitivity Vasculitis & Henoch-Schonlein Purpura in Children Iraj Salehi Abari MD., Internist Rheumatologist salehiabari@sina.tums.ac.ir

Hypersensitivity Vasculitis RRC-Amiralam Hypersensitivity Vasculitis Definition: Vasculitis of small blood vessels of the skin Due to an immune response or hypersensitivity reaction to an antigen Other names: Predominantly Cutaneous Vasculitis Cutaneous Leukocytoclastic Vasculitis I. Salehi A.,MD.

Pathology: Small blood vessels PMN nuclear debris; Leukocytoclasis RRC-Amiralam Pathology: Small blood vessels PMN nuclear debris; Leukocytoclasis Four histologic patterns: PMN or Mononuclear cell predominant Necrotizing or Non-necrotizing I. Salehi A.,MD.

Pathogenesis: Immune Complex process CIC, IC deposition RRC-Amiralam I. Salehi A.,MD.

Etiology: Antisera, serum sickness Idiopathic, most common Antigens: RRC-Amiralam Etiology: Antisera, serum sickness Idiopathic, most common Antigens: Drugs: Antibiotics, NSAIDs, Diuretic, Anticonvulsants Food: Cow’s milk, Chocolate, Cocoa Infection: HB or HC, Strep., M. TB., HIV Malignancy( Tumoral Ag ): Rare I. Salehi A.,MD.

Clinical manifestations: RRC-Amiralam Clinical manifestations: Palpable petechiae &/or purpura: Hallmark Lower extremities or dependent areas Other skin manifestations: Macules, Papules Vesicles, Bullae, Urticaria Subcutaneous nodules I. Salehi A.,MD.

Clinical manifestations: RRC-Amiralam Clinical manifestations: Other clinical findings: Fever, malaise, myalgia Arthralgia Lymphadenopathy Renal: uncommon, mild proliferative GN Liver, Lung, Heart, CNS: rare I. Salehi A.,MD.

Laboratory Data : No specific laboratory test Mild leukocytosis RRC-Amiralam Laboratory Data : No specific laboratory test Mild leukocytosis Low serum complement levels: C3, C4 Mild elevated ESR, CRP I. Salehi A.,MD.

Diagnosis: Hx.: Drugs, infections Ph. Ex.: Skin; Palpable purpura RRC-Amiralam Diagnosis: Hx.: Drugs, infections Ph. Ex.: Skin; Palpable purpura Pathology: small vessel vasculitis R/O HSP: No IgA deposition in skin or kidney Low serum complement level I. Salehi A.,MD.

Treatment: Discontinuation of the implicated drug RRC-Amiralam Treatment: Discontinuation of the implicated drug Treatment of the underlying infection In severe or persistent disease: Colchicine, NSAIDs, Dapsone In fulminant or progressive disease or other organ involvement: Corticosteroids + Cytotoxic agents I. Salehi A.,MD.

Henoch-Schonlein Purpura( HSP ) RRC-Amiralam Henoch-Schonlein Purpura( HSP ) Introduction: The most common vasculitis in children Ninety percent of cases occur in children The tetrad of clinical manifestations: Palpable purpura with Nl Plt. & Nl PT, PTT Arthritis/ Arthralgia Abdominal pain Renal disease I. Salehi A.,MD.

Epidemiology: Age: 3-15 y/o Incidence: 10-20 / 100,000 RRC-Amiralam Epidemiology: Age: 3-15 y/o Incidence: 10-20 / 100,000 Peak age of incidence: In 4 to 6 years 70 / 100,000 M/F ratios: 1.2-1.8 / 1 Black < White or asian Occurs in: Fall, winter, spring I. Salehi A.,MD.

Pathogenesis: Unknown Triggers: Leukocytoclastic + IgA1 ICs RRC-Amiralam Pathogenesis: Unknown Triggers: URT with Strep: 50% Vaccinations Insect bites Leukocytoclastic + IgA1 ICs Increased serum levels of IgA & IgA ICs I. Salehi A.,MD.

Clinical manifestation: RRC-Amiralam Clinical manifestation: Initial presentation: Lower limb palpable purpura: 75% Arthralgia/ arthritis: 15% The classic tetrad: Palpable purpura: almost all Arthralgia/Arthritis: 85% Abdominal colicky pain: 50% Renal disease: 20-50% I. Salehi A.,MD.

Skin manifestations: Erythematous, macular, or urticarial wheals RRC-Amiralam Skin manifestations: Erythematous, macular, or urticarial wheals Ecchymoses, petechiae & palpable purpura Rash; crops, symmetrical, gravity-dependent area Lower extremities Buttocks in toddlers Face, trunks, upper limb in nonambulatory children Edema in dependent, periorbital area in <3yr I. Salehi A.,MD.

RRC-Amiralam I. Salehi A.,MD.

RRC-Amiralam I. Salehi A.,MD.

Joints: Arthritis / Arthralgia Oligoarticular Transient or migratory RRC-Amiralam Joints: Arthritis / Arthralgia Oligoarticular Transient or migratory Nondeforming Lower limb, Large joints 1. Rash 2. Arthritis: common Arthritis without Rash: uncommon I. Salehi A.,MD.

GI. Manifestations: Occur in ½ of children RRC-Amiralam GI. Manifestations: Occur in ½ of children Mild: nausea, vomiting, abdominal pain, ileus Severe: GI hemorrhage, Ischemia & Necrosis, Perforation, Intussusception Guaiac( + )stool: 56% Massive GI hemorrhage: rare I. Salehi A.,MD.

GI manifestation: Intussusception: Rare GI manifest.: RRC-Amiralam GI manifestation: Intussusception: The most common GI manifest. Ileoileal Rare GI manifest.: Acute pancreatitis Gall bladder involvement Bowel perforation Protein-losing enteropathy I. Salehi A.,MD.

GI manifestation: 1. Rash 2. GI: 65-85% 1. GI 2. Rash: 15-35% RRC-Amiralam GI manifestation: 1. Rash 2. GI: 65-85% 1. GI 2. Rash: 15-35% GI without Rash: rare I. Salehi A.,MD.

Renal manifestation: Within initial 4 weeks; 20-54% RRC-Amiralam Renal manifestation: Within initial 4 weeks; 20-54% Hematuria &/or proteinuria Acute renal failure Nephrotic syndrome I. Salehi A.,MD.

Other organ involvement: RRC-Amiralam Other organ involvement: Scrotum: Epididymitis, Orchitis CNS & PNS Eyes: Keratitis & Uveitis: rare Respiratory tract: Impaired lung diffusion capacity: 97% Interstitial pneumonitis: 69% Pulmonary hemorrhage: rare I. Salehi A.,MD.

Recurrence: In 1/3 of cases Within 4 months of the initial episode RRC-Amiralam Recurrence: In 1/3 of cases Within 4 months of the initial episode Occur more commonly in: HSP with nephritis HSP with elevated ESR HSP need treatment with corticosteroid I. Salehi A.,MD.

Laboratory Data: Serum IgA level is elevated: 50-70% RRC-Amiralam Laboratory Data: Serum IgA level is elevated: 50-70% Leukocytosis + elevated ESR Nl Platelet count & Nl coagulation test U/A Serum Cr is checking if abnormal U/A Pathology: Skin: Leukocytoclastic V. + IgA Kidney: Mesangial IgA GN I. Salehi A.,MD.

Imaging studies: Plain abdominal X-Ray Abdominal Ultrasonography RRC-Amiralam Imaging studies: Plain abdominal X-Ray Abdominal Ultrasonography Barium enemas in intussusception rather than ileoileal Doppler US in scrotal involvement I. Salehi A.,MD.

Diagnosis: Clinical: Classic Biopsy: Incomplete or unusual RRC-Amiralam Diagnosis: Clinical: Classic Biopsy: Incomplete or unusual Pathology: Leukocytoclastic V. + IgA Renal biopsy: Dx. Is uncertain Severe renal involvement I. Salehi A.,MD.

Classification criteria: RRC-Amiralam Classification criteria: ACR criteria: Palpable purpura Age at onset < 20 years Acute abdominal pain Biopsy showing PMN in the walls of small arterioles &/or venules > 2 of 4  HSP; sen. & spec. # 90% I. Salehi A.,MD.

Classification criteria: RRC-Amiralam Classification criteria: EULAR & PReS consensus criteria( 2005 ): Palpable purpura + Nl Plt. + Nl coagulation test plus one or more of the following Diffuse abdominal pain Arthritis or arthralgia Any biopsy with predominant IgA deposition I. Salehi A.,MD.

Treatment: Outpatient: in the most cases NSAIDs: Naproxen RRC-Amiralam Treatment: Outpatient: in the most cases NSAIDs: Naproxen Corticosteroid: Prednisolone, Methylpred. Oral hydration Bed rest Symptomatic relief of joint & abdominal pain I. Salehi A.,MD.

Treatment: Hospitalization: Severe Renal involvement: RRC-Amiralam Treatment: Hospitalization: Severe Renal involvement: Acute Renal Failure( nephritic ) Significant proteinuria( nephrotic ) Renal Biopsy HDS + CTX Severe GI involvement: Intussusception GI bleeding HDS + Surgery I. Salehi A.,MD.

Prognosis: Excellent in children RRC-Amiralam Prognosis: Excellent in children In follow-up: screening for renal disease U/A BP A small minority develop: Renal complication GI complication I. Salehi A.,MD.

RRC-Amiralam I. Salehi A.,MD.