henoch schonlein purpura and kawasaki disease peter henning, do maj, mc, usa 2 december 2008
TRANSCRIPT
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Henoch Schonlein Purpura and Kawasaki Disease
Peter Henning, DOMAJ, MC, USA
2 December 2008
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Overview
• Summary• Epidemiology• Etiology and Pathogenesis• Clinical Manifestations• Diagnosis• Treatment
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Henoch Schonlein Purpura (HSP)• Most common systemic vasculitis in children• Etiology - Unknown• Pathogenesis – End organ IgA immune complex (IC) deposition • Diagnosis - Clinical
– Palpable purpura - Abdominal pain– Renal disease - Arthritis
• Complication - Renal• Treatment - Typically symptomatic
– Disease usually self limited – Unclear role of corticosteroids in TX– No specific agent proven efficacious for persistent renal disease
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Epidemiology• Disease of early childhood– 20/100K in UK children < 17 years-old– 70/100K in UK children 4-6 years-old– No comparable data in adults, less common
• Male : Female 1.2-1.8 : 1• Less common in African American children• More severe course in adults– More frequent and severe renal disease– Requirement for more aggressive treatment
• Seasonal variance; rare in summer
Arthritis Rheum 1997 May;40(5):859-64
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Etiology
• UNKNOWN• Precipitating antigen may be infectious– Many cases follow URI
• Twins following simultaneous adenovirus:– HSP in one, IgA nephropathy in other
J Pediatr 1985 Jan;106(1):27-32.
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Pathogenesis• Immune-complex mediated disease– IgA IC deposition within affected organs– Leukocytoclastic vasculitis of post capillary venules– IC of IgA1 ONLY subtype– Complexes activate complement (alternative)
• Hinge region O-linked glycans of IgA1 are deficient in galactose and/or sialic acid content– Renal mesangial cells bind galactose/sialic acid deficient hinge
regions• Berger’s disease (IgA nephropathy) also involves IgA1
exclusively
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Clinical Features
• Classic Tetrad (cumulative incidence)– Rash (100%)– Arthralgias (82%)– Abdominal pain (63%)
• GI bleeding (33%)– Renal disease (40%)
• Presenting feature by % – Rash 74%– Arthralgias 15%– Abdominal pain 12%
Medicine (Baltimore) 1999 Nov;78(6):395-409.
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Classification Criteria
ACR 1990• Palpable Purpura• Age at onset < 20 years• Actue abdominal pain• BX
– Granulocytes in walls of small arterioles / venules
• > or = 2 90% Sns / Spc
EULAR / PRES 2005• Palpable purpura WITHOUT
coagulopathy or PLTsAND
• Diffuse abd pain• Arthritis or arthralgia• BX with IgA deposition
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Palpable Purpura• Erythematous macules petechia / palpable purpura– NORMAL clotting studies and platelets
• Appears in crops• Symmetric distribution– Gravity / pressure dependent areas
• Dependent & periorbital edema in children < 3 • Palpable purpura DDX:– Mixed cryoglobulinemia– Hypersensitivity vasculitis
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Arthralgias
• Transient, migratory oligo• Knees and ankles > upper extremity joints• Non-destructive• Prominent periarticular swelling without
synovitis• Significant pain and limited use / ROM• DDX– JIA, RF, SLE
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Gastrointestinal
Mild• Nausea / vomiting• Collicky abdominal pain• Ileus
Severe• GI bleed• Bowel ischemia / necrosis• Intussusception
•Abd pain due to submucosal hemorrhage, edema•ABNL endoscopy, small bowel series
•Onset within 8 days of rash •Melana or hematochezia in 25%
•Occult bleeding in 50%
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Gastrointestinal
• Intussusception– Most common serious GI complication (3.5%)
• Limited to small bowel in 60% of cases• Initial diagnostic test– Ultrasound instead of contrast enema
• DDX– Appendicitis
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Renal Disease
• Renal involvement in 20-54% of HSP pts– 2 days to 4 weeks after onset of systemic symptoms
• Retrospective review of 261 pts– Micro hematuria 11% (n=37) – Gross hematuria 5% (n=12)– Concominant proteinuria 57% (n=28)• Most patients suffer only mild disease• Good prognosis– 21 / 1133 pts (1.8%) with renal impairment at 6wks - 36yrs
Chang WL. Ped Nephro 2005; 20(9):1269.
Narchi H. Arch Dis Child 2005; 90(9):916.
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Renal Disease• General correlation between disease severity and biopsy
findings• Asymptomatic hematuria: focal mesangial proliferation• Proteinuria: cellular proliferation• Nephrotic range proteinuria: crescents
• Percentage of glomeruli with crescents has prognostic significance
• >50% – 37% progressed to ESRD– 18% with CRI
• DDX– Berger’s Disease
J Am Soc Nephrol 1999 Dec;10(12):2637-44
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Clinical Findings in Adults with HSP
• Less common• Similar to children• Exceptions– Intussusception rare– Increased risk of renal involvement
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Diagnosis
• CLINICAL• CLASSIFICATION CRITERIA• Gold Standard: BIOPSY– Unusual presentation or significant renal disease– Adults due to decreased incidence– Typically skin or kidney– IgA deposition by immunofluorescence (IF)
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Skin Biopsy• Superficial dermis sufficient• BX < 24 hour-old lesion
– Older lesions with less specific changes• Leukocytoclastic vasculitis in post capillary venules• IgA deposition
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Renal Biopsy
• Reserved for patients with severe renal involvement• IgA deposition in mesangium– IgG, fibrin, C3
• Mesangial proliferation to crescentic GN– BX generally parallels clinical disease severity
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Additional Diagnostics
• Labs– NL coagulation studies, platelets• Due to DDX considerations
– Labs non specific– UA at DX and F/U
• Imaging– Plain films– Abdominal ultrasound
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Treatment
• Complete recovery 94% children, 89% of adults• Supportive TX in vast majority– Rest– Hydration– NSAIDS
• Hospitalization– Complications: GI, renal– Severe SXs: GI, dehydration, arthritis
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Treatment: Corticosteroids (CS)• Reported benefits
– duration abd pain, risk of recurrence, intussusception and renal involvement
• Literature review, 2007– CS may duration of abd pain and risk of persistent renal
disease– Significant limitations
• 2 RCT’s– 40 outpatients TX oral prednisone 2mg/kg x 1 week
• NO difference at one year in renal involvement– 171 hospitalized pts TX oral prednisone x 1 month
• NO difference in rate of renal involvement• HOWEVER greater resolution at 6 mos in those with renal involvement• abd and joint pain
• Further study neededWeiss PF. Pediatrics 2007; 120(5):1079.
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Treatment: Specific TX
• GI– CS not proven to decrease risk of intussusception
• Renal disease– No prospective studies
• Methylprednisolone pulse followed by oral (1mg/kg) x 3 months • CS and azathioprine• CS, cyclophosphamide, anticoagulation• Efficacies of plasmapheresis and IVIG are uncertain
– TXP: clinically evident recurrence in 35% of patients at 10 years
Medicine (Baltimore) 1999 Nov;78(6):395-409.
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Recurrence
• Reported in 1/3 of affected children– Usually within 4 months of presentation– Recurrences shorter, more mild– More common in pts with renal involvement
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Kawasaki Disease (KD)
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Kawasaki Disease (KD)• Mucocutaneous lymph node syndrome• 2nd most common childhood vasculitis• < 5 year old, Asian• Acute, self-limited medium-vessel vasculitis– Fever - Conjuctivitis– Rash - Mucositis– Extremity changes - LAD
• Complicated by coronary artery aneurysm (CA)• IVIG dramatic improves morbity / mortality– 4X prevalence of CA in pts TX with IVIG within 10
days
Furusho, K. Lancet 1984, 2(8411): 1055.
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Epidemiology
• 85% of cases in children < 5 years• Peak age 9-12 months• 3% in children < 6 months• Isolated case reports in adults• 1.5:1 male to female ratio• Genetic– 10x risk if affected sibling– 2x risk if affected parent
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Epidemiology
• Annual incidence varies– Japan ~100/100,000 children < 5 years• Increasing?
– South America – 3/100,000– US – 17/100,000 > 5 years• 9/100,000 among Caucasians• 17/100,000 among African-Americans• 33/100,000 among Asians
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Etiology• UNKNOWN• Genetic Factors
– High incidence among Asians and Asian-Americans– Relative risk of siblings of index case is 10 (Japanese data)
• Inositol 1,4,5-triphosphate 3-kinase (ITPKC)– Negative regulator of T-cell activation– Polymorphism of ITPKC assd with increased risk of KD
• Multiple HLA allele associations– B5, B44, Bw51, DR3 and DRB3*0301 in Caucasians– B54, Bw15 and Bw35 in Japanese– Bw51 in Israelis
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Etiology – Infectious Agent?
• Circumstatital supporting data– Similar clinical features to other infectious diseases– Seasonal peak in winter and spring– Geographically focal epidemics – Houoshold contacts (Japan) at increased risk– Peak incidence in toddler age group and rare cases in
infants < 3 months• Protective effect of transplacental antibodies?
• HOWEVER– NO substantiated specific infectious
association
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Pathogenesis
• Aberrant immune response– Activated macrophages
• Subendothelial inflammation• Transmural inflammation• Destruction of the media and aneurysm
formation
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Clinical Manifestations
• Fever • Bilateral conjunctivitis• Mucositis• Polymorphous rash• Extremity changes• Cervical LAD
• Arthritis• Lipid abnormalities• Renal/urinary findings• Vasculitis– Coronary artery
aneurysm– Cardiac complications
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Fever
• 100% of patients– Consider KD in children with unexplained fever > 5 days
• 38o to > 40o C• Persistent ; > 5 consecutive days– Untreated, usually lasts 1-2 weeks– Fever > 4 weeks, suspect other etiology
• Unresponsive to antibiotics, antipyretics
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Conjuctivitis• Bilateral• Seen in > 85% of patients• Onset within 2-4 days of fever onset• Non exudative• Blubar – spares the limbus• May present with acute, anterior uveitis, photophobia• Usually subsides within one week
Courtesy of Robert Sundel, MD.
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Mucositis
• 90% of pts• 2-5 days after fever onset• Discrete lesions NOT typical• Fissuring/cracking of lips• “Strawberry” tongue
Courtesy of Robert Sundel, MD.
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Name two other diseases in which you can see a “strawberry” tongue?
Scarlet fever and toxic shock syndrome
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Rash
• POLYMORPHOUS
• 80% of pts• Within 1-5 days of fever onset• Trunk and extremities• Frequently pruritic• Disappears when fever subsides
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Extremity Changes
• 70% of pts– Last SXs to develop
• Painful erythema hands / feet• Indurated edema• Desquamation• Disappear with resolution of fever• Arthritis
– 7.5-25% of cases– Oligo and poly of large joints– No prognostic difference b/t pts
with and without arthritis
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Eponym for transverse nail depressions (below) seen after KD?
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Eponym for these transverse nail depressions?
• Beau’s lines• Develop in response to many diseases (uncontrolled DM,
syphilis) including acute illnesses accompanied by high fevers. such as scarlet fever, KS, measles, mumps and pneumonia.
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Lymphadenopathy
• Least consistent feature– Absent in 50-75%
• Frequently unilateral• Usually anterior cervical– NOT generalized LAD
• > One lymph node>1.5cm
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Lab Findings
• Markers of inflammation– ESR, CRP, leukocytosis,
thrombocytosis
• Lipid ABNL– TG, LDL– HDL– NL with TX
• NC, NC anemia
• LFTs– Hepatic congestion
• CSF– Mononuclear
pleocytosis
• UA with WBCs
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Differential Diagnosis
• Most common DDX = exanthems of childhood• DX clues– Absence of fever– Presence of …. (SXs NOT consistent with KD)• EXUDATIVE conjunctivitis or pharyngitis• Bullous or vesicular rash• Generalized LAD
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Differential Diagnosis
• Viral– Measles, EBV, echo and adenovirus
• Toxin Mediated– Toxic shock syndrome, scarlet fever
• RMSF, leptospirosis• Drug Reaction– Steven’s Johnson
• Systemic JIA
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Cardiovascular Complications
• Major cause of morbidity and mortality– Coronary artery aneurysm (CA)– Coronary arteritis– Decreased intropy– Myocarditis and pericarditis– Mitral valve regurgitation (mild)
• Near universal coronary artery involvement• Infants < 1 year-old at increased risk• Treatment directed at preventing aneurysm
formation
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Coronary Artery Aneurysm
• 20-25% untreated vs. 4-13% treated• Findings associated with CA– Age < 1 or > 6 - Male– Fever > 14 days - Na+ < 135 mEq/L– HCT < 35% - WBC > 12K / mm3
• Prognosis dependant on size, shape– Best small (<8mm), fusiform (vs. saccular)
• Complications– Rupture - Thrombosis– MI - Stenosis after regression
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Coronary Artery Aneurysm
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Coronary Artery Aneurysm
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Other Complications
• Renal – rare aside from sterile pyuria– ARF due to multiple mechanisms
• GI – rare– Case series 10 patients, 5 gallbladder hydrops
• Macrophage activation syndrome– Case reports
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Diagnosis: Diagnostic Criteria
• Fever for > 5 days plus –
• Four of five following:– Bilateral conjunctivitis– Mucositis– Polymorphous rash– Extremity changes– Cervical adenopathy
• No other identifiable cause
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Diagnosis: Incomplete KD
• Definition– Presentation c/w KD but < 4 DX criteria
• Why is incomplete KD important?– CA risk– Delayed treatment– Poor prognosis for patients with incomplete KD
• Infants < 6 months at particular risk– Tend to have less complete presentation
Chang, FY. Infect Dis Jour. 2006; 25(3):241.
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AHA / AAP Guidelines: Evaluation of Incomplete KD
Newburger, JW. Pediatrics 2004; 114:1708.
Supplemental Lab Criteria:•Albumin 3.0• Anemia for age•Elevated ALT•PLT > 450K after 7 days•WBC >15K•UA with > 10 WBC / HPF
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Treatment - IVIG
• Only proven definitive therapy • Which patients should be treated?• Multiple risk (CA) scoring algorithms – none
validated• All patients DX with KD or incomplete KD
• Why? Efficacy; 6 RCTs, 1626 pts Therapy CA at 30 days CA at 60 days
ASA alone 26% 18%
IVIG 1gm/kg 16% 10%
IVIG 2gm/kg 4% 4%
Teraj, M. Jour Ped 1997; 131(6):888.
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Treatment - IVIG
• Dose: 2mg/kg over 8-12 hours– Despite dose response, lack of evidence > 2gm/kg– Studies support single infusion• CA, fever, length of hospitalizaion
• Administer during first 10 days of illness– Lack of studies TX after 10 days
• Effectiveness after 10 days?• Patients can be re-treated at same dose
Oates-Whitehead RM. Cochrane Db Syst Rev. 2003; (4):DC004000
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Treatment
• IVIG– Adverse Drug Effects - 1994 Hep C cases– $$ however cost / benefit analyses clearly favorable– Volume
• ASA– No benefit in reducing CA– All studies include ASA– Improves clinical, lab markers of inflammatory response
• Corticosteroids– Conflicting results from 2 multicenter RCTs
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Long-term Management
• Echo during acute phase and 6-8 weeks later– Risk stratification for MI and long-term complications
• Based on risk, AHA / AAP guidelines for– Medical TX (ASA, warfarin, LMWH)– Physical activity– Follow up schedule
• Vaccinations– Postpone all live virus (MMR, varicella) vaccines
for 11 months after IVIG
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Questions?
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Coronary Artery Aneurysm
• Regression– 50-70% regress spontaneously over 6 months to 2
years– Fusiform aneurysms more likely to regress than
saccular– Aneurysm size• Giant aneurysm (internal diameter >8mm) are less
likely to regress
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Coronary Artery Aneurysm
• Mechanism of regression– Inward migration and proliferation of smooth
muscle cells from the media layer– Proliferation of intimal cells– Persistently thickened intima– Residual endothelial dysfunction– Impaired myocardial perfusion
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Coronary Artery Aneurysm
• Rupture– Very rare– All documented cases have occurred during first
six weeks– Massive hemopericardium– Death
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Coronary Artery Aneurysm
• Thrombosis and recanalization– Increased platelet count– Enhanced platelet aggregation– Sluggish flow pattern– Arteriae in arteria
• Localized stenosis– Tend to progress– Ischemic heart disease
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Coronary Artery Aneurysm
• Myocardial infarction– Main cause of death in KS– Most deaths from AMI occur within one year of
disease onset– 37% with silent MI– 22% mortality with first event– 63% with second event– 83% with third event
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Cardiovascular Complications
• Signs and symptoms– Increased irritability, pallor– Cyanotic digits– Tachycardia, gallops, muffled heart sounds– Cardiomegaly– EKG changes• Prolongation of PR and QT intervals• Low voltage• ST-T wave changes
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Predictors of coronary artery aneurysm
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Evaluation of Coronary Artery Aneurysms
• Coronary angiography– Gold standard– Invasive– Expensive– Increased risk– Cannot define wall
pathology
• Other diagnostic tests– TTE– TEE– MRI/MRA– Intracoronary u/s
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Evaluation of Coronary Artery Aneurysms
• Transthoracic echocardiography– Primary technique– Readily available– Non-invasive– Sensitive in pediatric
population– Only proximal anatomy
seen
• Transesophageal echocardiography– Not as readily available– Invasive– More sensitive in adult
population– Better visualization of
coronary anatomy
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Evaluation of Coronary Artery Aneurysms
• Magnetic resonance imaging and angiography– Non-invasive– Limits in imaging – Difficulty in examining young patients
• Intracoronary ultrasonography– Wall pathology visualized– Highly invasive research tool
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