henoch schonlein purpura (iga vasculitis)

8/12/2019 Henoch Schonlein Purpura (IgA Vasculitis)

1/15

Benjamin Dowse, MD/PGY308/01/2014


2/15

3 year old previously healthy white female referred to the emergencydepartment from her PCP secondary to concern for a rash, whichbegan 4 days ago. She has also had intermittent fever since Saturday(Tmax 39.1 per parents, was afebrile in clinic). Has had NB/NB emesistoday and yesterday.

ROS: No diarrhea, no headache, fever improves with Tylenol perparents. Otherwise negative ROS.

HPI


3/15

Immunizations: UTD

NKDA

No routine home meds

Previously healthy, no chronic illness, hospitalizations or surgeries.

FHx: Mom with ankylosing spondylitis.

Past Medical History


4/15

VS: T 36.3, HR 108, R 24, Sat 98% RA, BP 90/60

General: Well developed 3 year old. Awake, alert, appropriate, non-toxic, no acutedistress. Cooperative, playful, talkative. No pain or discomfort.

HEENT: Normocephalic, atraumatic, PERRL, no conjunctival injection, TMs clear, narespatent, pharynx, mouth, tongue all without lesions, exudate or erythema, neck with noswollen or tender lymph nodes, supple with full range of motion.

Respiratory: Lungs CTAB, no increased WOB.

Cardiovascular: Normal S1/S2, without any murmur, gallop, click, or rub. 2+ pulses toupper and lower extremities. Capillary refill less than 2 seconds.

GI: Abdomen soft, non-tender. Normal bowel sounds, no organomegaly.

Musculoskeletal: No pain in extremities, normal range of motion without pain topassive or active movement. Mild pedal edema.

SKIN: Notable for purpuric type lesions. Predominantly to the lower extremities,but do extend to upper torso, both the front and the back. Also appear on her face,neck and arms. These lesions do not blanch. Small, approximately 1.5 cm atlargest. Non-painful.

Neurologic: Symmetric use of extremities, no evidence of weakness. Lower extremityreflexes symmetric, toes downgoing, no clonus, cranial nerves normal.

Physical Exam


5/15

3 yo previously healthy girl with fever, nausea/vomiting and diffusepetechial/pupuric lesions.

Differential Diagnosis


6/15

Infectious

Meningococcal/pneumococcal meningitis

Bacterial endocarditis

Viral hemorrhagic fevers

AIDS thrombocytopenia/HIV produced

Disseminated CMV

Ehrlichiosis

Measles (rubeola)

Rickettsial disease

Rocky mountain spotted fever

Babesiosis

Dengue Hemorrhagic fever

Colorado Tick Fever

Leptospirosis (Weils disease)

Rheum ITP

HSP

Neoplastic

AML/ALL

Lymphoma

Congenital

Kasabach-Merritt syndrome

FEN

Scurvy (vit C deficiency)

Genetic

Von Willibrandsdisease

Absent radius/thrombocytopenia syndrome

Wiskott-Aldrich Syndrome

Heme

Disseminated Intravascular coagulopathy

Aplastic anemia

Renal

Hemolytic Uremic syndome

Other

Heat stroke

Drug induced thrombocytopenia

Radiation exposure

Differential Diagnosis


7/15

CBC: WBC 10.3 (32% neut, 63% Lymph), HGB 12.4, Hct 35.3, PLTS 316

UA:

Normal color, appearance, glucose negative, hgb negative, protein trace, negative

nitrites and leuk esterase. Negative for bacteria, 3 WBC.

CMP:

Na 139, K 4.7, Cl 109, Bicarb 20, Glucose 73, BUN 13, Creatinine 0.35, Ca 9.5, Prot 6.5,

Albumin 3.5, Bili 0.3, Alk phos 237, ALT 10, AST 24

Labs


8/15

Most common form of systemic vasculitis in children.

Primarily between ages 3-15 years.

Highest incidence is 70 per 100,000 in children between four and sixyears.

About half of cases are preceded by a known upper respiratoryinfection.

Henoch Schonlein Purpura(IgA Vasculitis)


9/15

Immune-mediated vasculitis associated with immunoglobulin A deposition.

Underlying cause remains unknown, although immunologic, genetic, andenvironmental factors all seem to play a role.

Biopsies demonstrate involvement of small vessels (mostly post-capillary venules)

Pathophysiology


10/15

Immunofluorescence studies show IgA, C3, and fibrin deposition within vesselwalls. IgA, C3, fibrin, as well as IgG and IgM are deposited within the endothelialand mesangial cells of the kidney.

Pathophysiology

Immunofluorescence microscopy showing mesangialimmunoglobulin A (IgA) deposits


11/15

Clinical Features

Presenting symptoms:

Purpura 74%

Arthritis 15%

Abdominal pain 12%

Classic Tetrad

Palpable purpura without thrombocytopenia and

coagulopathy (all patients) (?)

Often begins as urticarial wheals which

coalesce and evolve into palpable purpura,

mostly in dependent areas.

Arthritis/Arthralgia (50-75% of patients)

Usually transient or migratory, one to four joints,

more commonly lower extremity joints. Abdominal pain (50% of patients)

Can develop intussusception, guaiac positive

stool, or just nausea. Endoscopy may

demonstrate purpuric lesions.

Renal disease (21-54% of patients)

Similar to IgA nephropathy. Watch for hematuria,

proteinuria. Occasionally can progress to serious

kidney disease.


12/15

This picture shows the classic skin manifestations of Henoch-Schnlein purpura (IgAvasculitis), with clusters of typical ecchymoses, petechiae, and palpable lesions on thelegs in a typical distribution (gravity/pressure-dependent areas).

Skin manifestations of Henoch-Schnlein

purpura (IgA vasculitis)


13/15

Usually clinically diagnosed. If unusual, biopsy of an affected organ can beconfirmatory.

Diagnosis


14/15

Follow-up Renal involvement

90% with renal involvement manifestwithin two months

96% within 6 months

Patients should be followed weekly

or biweekly for urinalysis and bloodpressure measurement for one totwo months after presentation

Then monthly for a year after initialmanifestation.

If persistent hypertension,proteinuria, or renal insufficiency,refer to nephrology.

Supportive care

Adequate rest, hydration,symptomatic relief of pain

Hospitalization?

Unable to hydrate

Severe abdominal pain

Significant GI bleeding

Altered mental status

Renal insufficiency (elevated

creatinine), hypertension, and/ornephrotic syndrome

Treatment


15/15

Gardner-Medwin JM, Dolezalova P, Cummins C, Southwood. Incidence of Henoch-Schnlein purpura, Kawasaki disease, and rare vasculitides inchildren of different ethnic origins. Lancet. 2002;360(9341):1197.

Rigante D, Castellazzi L, Bosco A, Esposito S. Is there a crossroad between infections, genetics, and Henoch-Schnlein purpura?Autoimm unRev. 2013 Aug;12(10):1016-21. Epub 2013 May 15.

Trapani S, Micheli A, Grisolia F, Resti M, Chiappini E, Falcini F, De Martino M. Henoch Schonlein purpura in childhood: epidemiological andclinical analysis of 150 cases over a 5-year period and review of literature. Semin Arthritis Rheum. 2005;35(3):143.

Trnka P. Henoch-Schnlein purpura in children.J Paed iatr Child Health. 2013 Dec;49( 12):9 95- 1003. Epub 2013 Oct 18.

Narchi H. Risk of long term renal impairment and duration of follow up recommended for Henoch-Schonlein purpura with normal or minimalurinary findings: a systematic review. Arch Dis Child . 2005;90(9):916.

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References

henoch schonlein purpura (iga vasculitis)

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