VASCULITIS SYNDROMES

VASCULITIS SYNDROMES
By:Emily B. Martin, MD
Rheumatology Board Review

KAWASAKI SYNDROME
Mucocutaneous lymph node syndrome
KAWASAKI DISEASE
* Diagnostic criteria:
CLINICAL MANIFESTIONS
* Arthritis and arthralgia
* Urethritis
* CNS involvement
* GI symptoms

DIFFERENTIAL DIAGNOSIS
* Viral infections
* Toxin mediated illnesses
* Rickettsial or spirochete infections
* Drug reactions
* JRA
* Mercury hypersensitivity reaction

LABORATORY EVALUATION
* Markers of systemic inflammation
* Anemia (normocytic, normochromic)
* Sterile pyuria (urethral origin, don’t do a cath)
* Transaminase elevation (mild to moderate)
* CSF findings
* Synovial fluid inflammation
* Hyponatremia (increased risk for coronary aneurysms)

TREATMENT
* Mainstay of treatment is IVIG 2 gram/kg over 8-12 hours.
* IVIG may need to be repeated in refractory cases.
* Several studies have shown that IVIG + aspirin decreases the risk of coronary aneurysms compared to aspirin alone.
* High dose aspirin during acute illness then low dose for about 2 months.

FOR THE BOARDS…
* Know the clinical manifestations of Kawasaki syndrome.
* Know the differential diagnosis of KD.
* Know the laboratory abnormalities seen in KD.
* Recognize the value of high-dose IVIG in treatment of KD.

QUESTIONS
HENOCH-SCHONLEIN PURPURA

* Most common systemic vasculitis in children.
* Immune mediated
* Often a self-limited disease.
* Occurs more often in fall, winter, and spring.
* About 50% of cases are preceded by URI’s.

CLINICAL PRESENTATION
* Classic tetrad
GI SYMPTOMS
* HSP can cause edema and submucosal hemorrhage of GI tract.
* May be the presenting symptom of HSP.
* Symptoms typically develop within 8 days of the rash.
* Intussusception is the most common GI complication.

RENAL DISEASE
* Occurs in up to 50% of patients.
* Ranges from hematuria to end-stage renal disease (<1% of patients).
* Usually presents within four weeks of onset of HSP.
* Overall prognosis is very good, but there is some long-term risk of progressive renal impairment.

LABORATORY FINDINGS
* There is NO definitive diagnostic test.
* IgA levels may be elevated in 50-70% of patients.
* Platelet counts and coag studies should be normal.
* Inflammatory markers may be elevated.
* Urinalysis
* Negative RF and ANA.
* Recognize the typical presentation of HSP.
* Recognize that HSP may present initially with ABDOMINAL PAIN OR JOINT COMPLAINTS.
* Know the typical laboratory findings in HSP.

MOST likely diagnosis is
* Henoch-Schonlein purpura
* Immune thrombocytopenic purpura
* Juvenile rheumatoid arthritis
* Parvoviral infection
* Post-streptococcal arthritis

VASCULITIS SYNDROMES.ppt