• Clinical and Experimental Pediatrics
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• 제63권5호
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• pp.158-163
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• 2020

IgA vasculitis, formerly known as Henoch-Schönlein purpura, is a systemic IgA-mediated vasculitis of the small vessels commonly seen in children. The natural history of IgA vasculitis is generally self-limiting; however, one-third of patients experience symptom recurrence and a refractory course. This systematic review examined the use of dapsone in refractory IgA vasculitis cases. A literature search of PubMed databases retrieved 13 articles published until June 14, 2018. The most common clinical feature was a palpable rash (100% of patients), followed by joint pain (69.2%). Treatment response within 1-2 days was observed in 6 of 26 patients (23.1%) versus within 3-7 days in 17 patients (65.4%). Relapse after treatment discontinuation was reported in 17 patients (65.4%) but not in 3 patients (11.5 %). Four of the 26 patients (15.4%) reported adverse effects of dapsone including arthralgia (7.7%), rash (7.7%), and dapsone hypersensitivity syndrome (3.8%). Our findings suggest that dapsone may affect refractory IgA vasculitis. Multicenter randomized placebo-controlled trials are necessary to determine the standard dosage of dapsone at initial or tapering of treatment in IgA vasculitis patients and evaluate whether dapsone has a significant benefit versus steroids or other medications.

• Childhood Kidney Diseases
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• 제24권1호
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• pp.1-13
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• 2020

Immunoglobulin (Ig)A vasculitis nephritis (IgAVN), also referred to as Henoch-Schönlein purpura nephritis, is a relatively benign disease in children. However, two 24-year European cohort studies have reported high sustained rates of hypertension, severe proteinuria, and renal dysfunction in patients with IgAVN. Notably, the incidence and exacerbation rates of proteinuria, hypertension, and renal dysfunction during pregnancy were high even in women who recovered from IgAVN before pregnancy. Patients with IgAVN need lifelong care. Trials have been performed to investigate early biomarkers and genes associated with poor prognosis to identify high-risk patients in whom IgAVN may progress to severe renal disease. Urinary IgA/cr, IgM/cr levels, and HLAB35 and angiotensinogen gene expression were shown to be predictors of progression of IgAVN to severe renal dysfunction. The 2019 Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) initiative group published guidelines for pediatric IgAVN, following the Kidney Disease: Improving Global Outcomes (KDIGO) guidelines established in 2012. Compared with the KDIGO guidelines, the SHARE guidelines recommend earlier corticosteroid administration in cases of mild proteinuria (>0.5 g/d). Clinical trials of targeted budesonide delivery to the distal ileum, monoclonal antibody targeting C5, eculizumab and anti-CD20 monoclonal antibody administration, among others are currently underway in patients with IgA nephropathy. It is expected that newer therapeutic agents would become available for IgAVN in the near future. This review summarizes IgAVN with emphasis on recently published literature, including possible preventive strategies, predictive biomarkers for progression of IgAVN, and various treatments.

• Tuberculosis and Respiratory Diseases
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• 제39권1호
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• pp.89-94
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• 1992

저자들은 전신성홍반성낭창에서 신장염에 이어 급성 폐렴이 발생하여 이의 악화로 사망한 환자를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• Childhood Kidney Diseases
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• 제10권1호
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• pp.40-44
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• 2006

[ $Henoch-Sch{\"{o}}nlein$ ] 자반증은 소혈관의 혈관염으로 오는 전신적 혈관 장애로 자반, 위장 증세, 관절 증세, 신 증세를 주 증세로 하는 질환이다. HSP의 흔한 피부증상은 주로 궁둥이와 하지의 자반 증상이며 성인에서는 출혈성 수포를 동반하는 경우가 흔하나 소아에서는 드문 것으로 알려져 있다. 저자들은 관절통과 복통을 호소하는 환아에서 출혈성 수포가 동반되어 있어 피부조직 검사를 하였고 진피의 leukocytoclastic vasculitis와 면역 형광법상 소혈관의 IgA와 fibrinogen의 침착을 관찰하였다. HSP로 진단하여 스테로이드 치료를 하였고 수일 내로 관절통과 복통이 호전되어 문헌고찰과 함께 보고하는 바이다.

• Clinical and Experimental Vaccine Research
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• 제13권2호
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• pp.166-170
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• 2024

The coronavirus disease 2019 (COVID-19) vaccine was developed to provide immunity against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), which was first reported in 2019. The vaccine has proven to be effective in reducing severity and mortality and preventing infection. Henoch-Schönlein purpura is an autoimmune vasculitis (immunoglobulin A vasculitis). Historically, vaccines have been administered primarily to children, and Henoch-Schönlein purpura has often been reported in children following vaccination. However, since the start of COVID-19 vaccination, an increasing number of cases have been reported in adults. Here, we report a case of a patient who developed hematuria and proteinuria after receiving the messenger RNA COVID-19 vaccine. A 22-year-old man presented to the hospital with a lower extremity rash, bilateral ankle pain, and abdominal pain 18 days after receiving the COVID-19 vaccine. The man had no significant medical history and was not taking any medications. Laboratory tests showed normal platelet counts but elevated white blood cell counts and C-reactive protein and fibrinogen levels. He was treated with the non-steroidal anti-inflammatory drugs, pheniramine and prednisolone. At 40 days after starting treatment, C-reactive protein levels were within normal limits, and no hematuria was observed. Treatment was terminated when the purpura disappeared. This report is intended to highlight the need for further research to be proactive and carefully monitor for conditions associated with the COVID-19 vaccine.

• Parasites, Hosts and Diseases
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• 제60권2호
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• pp.133-137
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• 2022

Toxocariasis is one of the most common geohelminth infections in several parts of the world. We describe a rare case of ocular toxocariasis with secondary exudative retinal detachment treated with albendazole and an intravitreal dexamethasone implant. A 13-year-old boy with counting finger vision was diagnosed with retinal vasculitis and exudative retinal detachment in his right eye. Fundoscopic examination revealed retinal hemorrhage, retinal vasculitis, and exudative retinal detachment. Serological test using serum and intraocular aqueous humor were positive for anti-Toxocara specific IgG antibodies. He received repeated doses of intravitreal dexamethasone implants combined with oral albendazole. A sequential follow-up optical coherence tomography revealed that the retina was successfully reattached. His visual acuity subsequently improved to 20/400.

• Journal of Yeungnam Medical Science
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• 제25권1호
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• pp.58-63
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• 2008

Henoch-Sch$\ddot{o}$nlein purpura (HSP) is a leukocytoclastic vasculitis of small vessels with deposition of IgA, commonly resulting in skin, joint, gastrointestinal, and kidney involvement. HSP is an uncommon disorder in adults and accounts for 0.6% to 2% of adult nephropathy. We report a case of HSP with acute renal failure successfully treated with corticosteroid. In this case, the patient presented with vasculitic purpuric rash on lower extremity, arthralgia in the wrist, abdominal pain, hematochezia, oliguria and azotemia. Abdominal CT showed wall thickening of the small and large bowels. Skin biopsy revealed leukocytoclastic vasculitis. Percutaneous renal biopsy showed no crescent formation, but mesangial IgA and $C_3$ deposits were observed by immunofluorescence. The patient was treated with corticosteroid (1mg/kg per day) and hemodialysis. After treatment, renal function improved and purpuric lesion, arthralgia and abdominal pain disappeared. Thus, when adults present with purpuric rash and rapidly progressive glomerulonephritis (RPGN), HSP should be a diagnostic consideration.

• Pediatric Infection and Vaccine
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• 제4권1호
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• pp.140-149
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• 1997

목 적 : 가와사끼병은 주로 5세 이하의 소아에서 많이 볼 수 있는 고열을 주소로 하는 신체내 어느 장기에나 혈관염을 일으키는 질환으로, 원인은 아직 밝혀지지 않았지만 어떤 감염원에 의하거나, 또는 감염균에 의해 생성된 항원에 의한 인체내 면역반응의 이상으로 생긴 자가 면역질환의 하나일 가능성이 제시되고 있어, 이에 저자들은 환아의 혈청내 면역 글로부린중 IgG 아형항체가를 급성기 고열시기에 측정하여 정상치와 비교하여 봄으로써 이 병의 면역학적 변화를 관찰하고, 아울러 진단과 치료에 도움이 되고자 본 연구를 시행하였다. 방 법 : 1995년 1월부터 1996년 12월까지 본원 소아과에 입원한 급성기 고열이 있던 가와 사끼병 환아 35명을 대상으로 혈청을 채취하여 Enzyme Immuno Assay(EIA) 법을 이용하여 총 IgG, IgM, IgA, IgE를 측정하였고, Single Radial Immuno Diffusion(SRID)법을 이용 하여 IgG 아형항체가를 측정하여 연령별 정상치와 비교하여 보았다. 결 과 : 1) 남여 비는 1.5:1.0으로 남아에서 많았다. 2) 총 IgG, IgM, IgA, IgE 는 대부분 연령에 따른 정상치 범위내에있었다. 3) 모든 환아에서 CRP, ESR, C3 등이 증가하는 급성기 염증반응을 볼 수 있었고 백혈구 증다증을 보인 경우가 많았다. 4) IgG 아형항체 중 IgG1, IgG2, IgG3는 연령별 정상치 범위내에 있었으나, IgG4는 모든 환아에서 현저하게 감소되어 있었다. 결 론 : 현재까지 알려진 바로는 원인과 병리기전이 정확히 밝혀지지는 않았지만, 가와사끼병이 감염원에의한 면역 반응의 이상으로 생긴 자가 면역질환의 일종일 가능성이 제시되고 있으므로 낮은 IgG4 치가 면역반응에 영향을 주어 이 병의 이환율과 합병증 발생의 위험성을 높일 수 있다고 생각되나, 각 보고자마다 결과가 서로 달라 좀 더 많은 시간과 예를 모아 연구 할 필요가 있다고 사료된다.

• Tuberculosis and Respiratory Diseases
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• 제67권3호
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• pp.234-238
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• 2009

Henoch-$Sch\ddot{o}nlein$ Purpura (HSP) is a systemic vasculitis involving small vessels with the deposition of immune complexes containing IgA and C3, which is characterized by associated skin, joint, renal, and gastrointestinal manifestations. Although there were several causes such as autoimmunity, infection, and drugs, it also can be presented as a form of vasculitidies associated with malignancies. We report a case of HSP developed during treatment for non-small cell lung cancer in a 66-year-old man. Multiple purpuric skin lesions occurred in both legs after the first cycle of chemotherapy, which was diagnosed as HSP by clinical and pathologic examinations. Due to the itching sensation, topical steroid was applied and the patient was improved 3 weeks later without a scheduled change in chemotherapy.

• Tuberculosis and Respiratory Diseases
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• 제63권6호
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• pp.531-536
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• 2007

웨게너 육아종증은 중형내지 소형의 동맥과 정맥을 침범하는 전신적 혈관염의 한 형태로 상, 하부 기도 및 신장의 질환을 특징으로 한다. 신장에 국한된 질환으로 나타날 때에는 Henoch-$Sch\ddot{o}nlein$ 자반증, 현미경적 다발성 혈관염 등의 기타 신장 침범 혈관염과 구분하기 어렵다. 본 증례에서는 초기 신조직검사에서 IgA 양성으로 Henoch-$Sch\ddot{o}nlein$ 자반증으로 진단되고, 15년 후에 폐조직 검사에서 웨게너 육아종증으로 진단된 1예를 경험하였기에 문헌고찰과 함께 보고한다.