Henoch Schonlein Purpura in Childhood: Epidemiological and Clinical Analysis of 150 Cases Over a 5-year Period and Review of Literature
Section snippets
Patients and Methods
This is a retrospective data study of all children with HSP hospitalized at the Anna Meyer Children’s Hospital, Florence, Italy, from January 1998 through December 2002; they were selected by a computerized search based on the discharging diagnosis of HSP.
Epidemiological Features
The main epidemiological data and etiological factors are shown in Table 1. Between January 1998 and December 2002, 150 white children, fulfilling the inclusion criteria described above in the “Patients and Methods” section, were selected. The patient population included 95 boys (63%) and 55 girls (37%). Males were affected more often than females (ratio M:F = 1.8:1). The patients ranged in age from 1 to 15 years (mean age 6.1 ± 2.7 years, median age 5.3 years). The age and gender distribution
Clinical Features
The main clinical manifestations of the 150 patients are summarized in Table 2.
Laboratory Findings
The main laboratory data of the acute phase are shown in Table 4. An increased ESR was detected in 85 (57%) patients. Leukocytosis was present in 31 (21%) patients, anemia in 21 (14%), and increased serum AST and/or ALT level in 5 (3%). Serum creatinine was measured in 145 patients and was normal in all but the 3 patients with renal insufficiency. Serum IgA level, evaluated in 104 patients, was higher than the normal range in 22 of 43 (51%) children less than 5 years old, and in 16 of 61 (26%)
Treatment
Twenty-two patients (15%) received nonsteroidal antiinflammatory drugs (NSAIDs), ie, flurbiprofen (5 mg/kg per day) or naproxen (15 mg/kg per day), to reduce severe joint pain. The duration of NSAID treatment ranged from 3 to 12 days (mean 6.1 ± 2.3 days).
A total of 19 children (13%) received corticosteroids, due to severe abdominal involvement in 16 and severe nephropathy in 3 cases. These patients were treated with oral or intravenous prednisone or prednisolone (1 to 2 mg/kg per day). The
Outcome
One year after data collection, the patients’ families were interviewed by telephone. The parents of 118 children (79%) provided information about the outcome of the disease. After at least 1-year follow-up (range 1 to 6 years, mean follow-up 2.5 years ± 0.8, median 2.1 years), 98 of 118 (83%) patients were found to have recovered within 2 weeks, and 20 (17%) patients recovered within 2 months after disease onset. Mean disease duration was 3 weeks. In 41 (35%) patients there had been a
Discussion
To our knowledge, this study provides 1 of the most extensive epidemiological reports published on HSP in children. In the present study, 150 children who presented with HSP over a period of 5 years were analyzed. HSP is the most common acute vasculitis affecting children, although it has also been reported in adults, especially in association with malignancies (23, 24, 25). Two cases of children with HSP and Wilm’s tumor have been published (26). The annual incidence of HSP ranges from 10 to
References (100)
Influence of age on Henoch Schonlein purpura
Lancet
(1997)- et al.
Association of Henoch Schonlein purpura glomerulonephritis with C4 B deficiency
J Pediatr
(1990) - et al.
Complement phenotypes in glomerulonephritisincreased frequency of homozygous null C4 phenotypes in IgA nephropathy and Henoch Schonlein purpura
Kidney Int
(1984) - et al.
Comparative clinical and epidemiological study of hypersensitivity vasculitis versus Henoch Schonlein purpura in adults
Semin Arthritis Rheum
(1999) - et al.
Henoch Schonlein purpura in children and adultsclinical differences in a defined population
Semin Arthritis Rheum
(2002) - et al.
Adult Henoch Schonlein purpura associated with malignancy
Semin Arthritis Rheum
(2000) - et al.
Incidence of Henoch Schonlein purpura, Kawasaki disease and rare vasculitides in children of different ethnic origins
Lancet
(2002) - et al.
Group A streptococcal antigen in the glomeruli of children with Henoch Schonlein nephritis
Am J Kidney Dis
(2003) - et al.
Chronic parvovirus B19 infection and systemic necrotizing vasculitisopportunistic infection or etiological agent?
Lancet
(1994) - et al.
Leucocytoclastic vasculitis after pneumococcal vaccination
Am J Infect Control
(1998)
Acute hemorrhagic edema of infancy (AHEI)—a variant of Henoch Schonlein purpura or a distinct clinical entity?
J Allergic Clin Immunol
Schonlein-Henoch syndrome in adults and children
Semin Arthritis Rheum
Henoch Schonlein purpura
Adv Pediatr
Henoch Schonlein purpura
Lancet
Pulmonary involvement in HSP
Mayo Clin Proc
Renal involvement in Schonlein-Henoch purpuraa multivariate analysis of prognostic factors
Kidney Int
The adult kidney 24 years after childhood HSPa retrospective cohort study
Lancet
Plasmapheresis as the sole therapy for rapidly progressive Henoch Schonlein purpura nephritis in children
Am J Kidney Dis
Commentarii di morborium historia et curatione. London: Payne, 1801
Uber eine eigenthumlike Form von Purpura
Berl Klin Wochenschr
Leukocytoclastic vasculitis
Childhood vasculitis
Lupus
Lehrbuch der sauglingskrankheiten
Auff Edit Amsterdam
In vivo activation of complement by IgA in a rat model
Clin Exp Immunol
An acute model for IgA-mediated glomerular inflammation in rats induced by monoclonal polymeric rat IgA antibodies
Clin Exp Immunol
Low serum C3, leukopenia, and thrombocytopeniaunusual features of Henoch Schonlein purpura
Eur J Pediatr
Adhesion molecules in autoimmune disease
Semin Arthritis Rheum
Adhesion molecules
A rheumatologic perspective. Arthritis Rheum
Tumor necrosis factor induced adhesion molecule serum concentrations in Henoch Schonlein purpura and pediatric Systemic Lupus Erythematosus
J Rheumatol
Henoch Schonlein purpura in childrenreport of 100 patients and review of the literature
Medicine
Risk factors on renal involvement and significant proteinuria in Henoch Schonlein purpura
Eur J Pediatr
Henoch Schonlein purpura in children from Northwestern Spain
Medicine (Baltimore)
The American College of Rheumatology 1990 criteria for the classification of Henoch Schonlein purpura
Arthritis Rheum
Hypersensitivity vasculitis and HS purpuraa comparison between the 2 disorders
J Rheumatol
Anti-streptolysin O and anti-deoxribonuclease B titersnormal values for children ages 2 to 12 in the United States
Pediatrics
Vasculitis as a paraneoplastic syndromereport of 11 cases and review of the literature
J Rheumatol
Cutaneous leucocytoclastic vasculitisa clinical and aetiologic study
Br J Rheumatol
Occurrence of HSP in a child with Wilm’s tumor
Med Pediatr Oncol
Long term renal prognosis of Henoch Schonlein purpura in unselected childhood population
Eur J Pediatr
Henoch Schonlein purpura in Arab children
Analysis of 52 cases. Trop Georg Med
Epidemiology of a cluster of Henoch Schonlein purpura
Am J Dis Child
Epidemiology of Schonlein-Henoch purpura
Acta Pediatr Scand
Incidence of vasculitis in children in the Czech republic2-year prospective epidemiology survey
J Rheumatol
[Henoch Schonlein Syndromeclinical and epidemiological analysis of 98 cases]
Med Surg Ped
[Retrospective analysis of Henoch Schonlein purpura in childrendiagnostic difficulties and renal outcome]
] Med Surg Ped
Cutaneous vasculitis in children and adultsassociated diseases and etiologic factors in 303 patients
Medicine
Neonatal acute hemorrhagic edema of childhoodcase report and review of the English-language literature
Pediatr Dermatol
Acute hemorrhagic edema of infancya case report
Clin Exp Dermatol
Acute hemorrhagic edema of infancy
Cutis
Acute hemorrhagic edema of the skin in infancy
Eur J Dermatol
Cited by (332)
Pediatric rheumatic diseases
2023, Encyclopedia of Child and Adolescent Health, First EditionFrontal region oedema as the first sign in Henoch-Schönlein purpura
2023, Anales de PediatriaVasculitis in children: A diagnostic challenge
2022, Journal de Pediatrie et de PuericultureVasculitis and Kidney Disease
2022, Pediatric Clinics of North America