Initial manifestations and short term follow-up results of Henoch-Schönlein purpura in children: report from two centers.

Abstract

H enoch-Schönlein purpura (HSP), also known as IgA vasculitis, is the most common form of systemic vasculitis in children [1]. HSP is grouped under the rubric of small vessel vasculitis and typically involves skin, joints, kidneys and gut. The clinical spectrum may range from mild skin rash and arthralgia to severe gastrointestinal (GI) and renal involvement [1, 2]. All of the patients have a purpuric rash typically located on the legs and buttocks. The rash may also extend to the arms and face [1-3]. Sometimes, GI manifestations, such as abdominal pain or join manifestations like arthralgia or arthritis, may precede the development of a purpuric rash in less than 5% of the patients [3]. Other manifestations are low-grade fever, lethargy, myalgia, orchitis, sub-ABSTRACT OBJECTIVE: This retrospective observational study aims to demonstrate initial signs and symptoms of Henoch-Schönlein purpura (HSP), search for risk factors for gastrointestinal and renal involvement and give short term follow-up results. METHODS: The files of newly diagnosed HSP patients from two pediatric rheumatology centers in the southeastern part of the country were retrospectively analyzed in this study. Demographic, clinical features and laboratory results were recorded from the files. RESULTS: The cohort consisted of 323 children (males: 53.6%, females: 46.4%). Median age at the time of diagnosis was 7.5 (1.8-17.3) years and the median duration of follow-up was six (3-22) months. The rash was present in all cases but was not the first symptom in 22.9% of the cases. Arthritis and abdominal pain before the development of rash were the initial symptoms in 11.8% and 11.1% of the cases, respectively. Other manifestations were subcutaneous edema (63.2%), arthralgia (57.6%), arthritis (27.6%), myalgia (17.6%), lethargy (10.2%), orchitis (7.5%) and fever (5.3%). Gastrointestinal involvement was seen in 53.3% and renal involvement in 23.5% of the cases. None of the patients developed renal impairment during the follow-up. Older age at diagnosis, necrotic rash, subcutaneous edema, abdominal pain, lethargy, myalgia, arthralgia and arthritis were significantly higher in patients with renal involvement, but none of the demographic, clinical and laboratory features was an independent risk factor for renal or gastrointestinal involvement. CONCLUSION: Abdominal pain, arthritis may be the first manifestation of HSP. Having constitutional symptoms, such as fever, myalgia and lethargy at the time of diagnosis, may be warning signs of a more aggressive course with gastrointestinal and renal involvement.

Henoch Schönlein Purpura / Ig A Vasculitis in Children and Risk Factors for Renal Involvement

“…Compatible with recent studies from our country, which showed a disease onset between 7.4-9.3 years, we found that the mean age at diagnosis was 8.49 ± 3.35 years [14][15][16] in our patient population. .…”

Clinical and laboratory features of IgA vasculitis with gastrointestinal involvement: a 12-year experience of the Referral Centre for Paediatric and Adolescent Rheumatology of the Republic of Croatia

“…bolesnici sa zahvaćenim gastrointestinalnim sustavom imali su veću pojavu generaliziranoga purpuričnog osipa, odnosno purpure proširene na oba ekstremiteta (14-16). Klinički najznačajniji rezultati vezani su uz činjenicu da su bolesnici u kojih su se pojavili gastrointestinalni simptomi u konačnici češće razvili nefritis u sklopu igav-a, što je najvažnija kronična komplikacija bolesti koja može rezultirati kroničnim zatajenjem bubrega u 1-15% bolesnika (17)(18)(19)(20). u našoj kohorti bolesnika učestalost nefritisa bila je 1,81 puta veća u skupini bolesnika koji su imali barem jedan gastrointestinalni simptom.…”

“…druga povezanost između gastrointestinalnog sustava i bubrega očitovala se u većoj učestalosti mikrohematurije u bolesnika s gastrointestinalnim simptomima, a ti su bolesnici češće liječeni glukokortikoidima, imunosupresivima i antihiper tenzivima te su imali dulje trajanje hospitalizacije (p<0,001) (4,12). povezanost zahvaćanja probavnog sustava i bubrega u igav-u opisana je u nekim istraživanjima, ali rezultati nisu jednoznačni (13,18,21,22). patofiziološki mehanizam kojim bi se objasnila povećana učestalost nefritisa u bolesnika koji su imali prisutne gastrointestinalne simptome nije poznat.…”

An overview of Henoch Schonlein Purpura Diagnosis and Management Approach

“…Rarely, HSP patients may develop central nervous system symptoms and signs, and in these cases, magnetic resonance imaging is useful for the assessment of these abnormalities. [15] Moreover, a thorough work-up of renal functions is usually done in these patients, and a renal biopsy is indicated when an unrelenting nephrotic syndrome is present. Patients with HSP usually have crescentic glomerulonephritis changes which signify a severe disease.…”

“…This is because HSP patients lack a classical complement pathway activation, a finding that is not characteristic of any other autoimmune inflammations of the glomerulus. [11,14,15]…”