CC March 2016 Henoch-Schonlein purpura (HSP)

echoecho's version from 2016-05-31 02:03


Question Answer
Henoch-Schonlein purpura (HSP) is the most common vasculitis of childhood with an estimated incidence of ____/100,000 children < 17 years of age?20
The majority of cases occur in children < ____ years of age with a mean age at dx of ____ years?10; 6
Incidence is ____ cases/100,000 in the 4-6 year age group?70
It can less commonly affect what 3 categories of patients?infants, adolescents, adults
What gender is affected twice as often than the other gender?males are affected twice as often as females
What two ethnic groups are affected more than AA?whites and Asians
List the HSP diagnostic criteria?Palpable purpura and at least ONE of the following = 1) abdominal pain 2) any biopsy specimen showing predominantly IgA deposition 3) arthritis or arthralgias 4) renal involvement evidenced by hematuria or proteinuria
Describe the what location do HSP skin lesions show up on the body?nonpruritic and located in dependent areas that are subject to pressure (lower extremities and buttock)
Because of infants spend less time upright and are not weight-bearing, what do the lesions locate at?at the face, upper extremities and upper body
Describe the lesion progression of HSP?1) start as erythematous papules or urticarial wheals 2) then progress to petechiae, palpable purpura and ecchymoses 3) lesions come in crops and progress from red to a rust color before fading over 1-2 weeks 4) non-pittine edema may also be present especially in young children and infants
Abdominal pain is present in up to ___% of patients?75
Describe the abdominal pain?colicky and diffuse
What is the effect of IgA vasculitis in the bowel results?results in mucosal edema and both accult and overt upper and lower tract bleeding (up to 50% of cases)
Intussusception may occur with a ____ _____ or ___ ___ acting as the lead point?mural hematoma; bowel edema
Joint involvement is seen in ___ to ___% of cases?75-80%
Which joints are most commonly affected? What is the symptoms?1) knees and ankles 2) swelling, pain, warmth and tenderness
The joint symptoms are transient and may precede the ____ and are not associated with any long-term permanent deformity?rash
Renal involvement is the most serious manifestation of HSP, affecting up to ___% of patients and resulting in morbidity including chronic kidney disease and rarely, mortality?50
Unlike the rash, renal involvement tends to be a later manifestation, usually occurring in the ___ month, but onset may be delayed?first
97% of renal involvement will manifest by ____ months?6
Screening urinalysis and BP measurement may identify ______ renal involvement?subclinical
Disease severity can range from microscopic hematuria (which,w hen alone, usually resolves w/o long-term sequelae) to what?to crescentic glomerulonephritis with heavy proteinuria
The heavier the proteinuria means what?the worse the prognosis and the greater the chance of progression to CKD
Serial UA are indicated to monitor what?development of renal involvement
List the less common symptoms?1) CNS (vasculitic encephalopathy) 2) scrotal involvement (pain and swelling) in boys
Adults may have what manifestations?1) pulmonary with interstitial disease 2) alveolar hemorrhage
Unlike Kawasaki disease, is there cardiac involvement?no
List the 7 differential dx of HSP?1) bacterial endocarditis 2) child abuse 3) Kawasaki disease 4) leukemia 5) meningococcemia 6) polyarteritis nodosa 7) Rocky Mountain spotted fever
The diagnosis of HSP is ______? Is there any diagnostic tests to dx HSP?clinical; no (CBC is typically nnormal, including platelets. Serum IgA levels may be elevated but do not correlated with disease severity. ESR is elevated in only 1/2 of patients. Rheumatologic testing like ANA, complement levels may help to identify other disease in the diff dx. Renal function testing at baseline is important, coagulation tests will help to r/o bleeding diatheses)
Spontaneous resolution of symptoms occur in ____% of children and _________% of adults?94; 84
Treatment?supportive with relative rest and NSAIDs for joint and abdominal pain (NSAIDs may aggravate GI s/s and should be used with caution in those with renal involvement)
In most cases, outpatient treatment is appropriate. In patient treatment is indicated when?1) severe abdominal pain 2) hemorrhage 3) dehydration 4) absence of adequate home monitoring
*** What may be used in cases of joint and abdominal pain not responsive to NSAIDS?corticosteroids (usually administered IV as these patients are usually hospitalized)
*** Do corticosteroids prevent renal disease?no
Eisting evidence supporting the use of immunosuppressants (cyclophosphamide, calcineurin inhibitors) alone or in combination with corticosteroids for HSP and renal involvement is ______ and of poor quality?inconsistent
Relapse will occur in ____ of patients?1/3
For all patients, long-term follow- is important, why?as complications of HTN and CKD may occur as long as 10 year later
*** SUMMARY = HSP is the most common vasculitis of childhood with mean age at diagnosis of ___ years?6
*** SUMMARY = Palpable purpura is required for the dx along with at least ONE other finding, list these?1) abdominal pain 2) IgA deposition on biopsy specimen 3) arthritis / arthralgia 4) hematuria or proteinuria
*** SUMMARY = Is there specific dxtic tests for HSP?no
*** SUMMARY = Renal involvement can occur up to ___ days later, so long-term follow-up is necessary?10