• Clinical and Experimental Pediatrics
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• v.63 no.5
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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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• v.24 no.1
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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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• v.39 no.1
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• pp.89-94
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• 1992

A 16 years old girl with systemic lupus erythematosus had a high fever for 20 days. Skin and renal biopsy showed diffuse granular deposits (IgG, IgM, $C_3$, $C_{1q}$ at dermo-epideral junction and IgG, IgA, IgM, $C_3$, $C_{1q}$, fibrinogen in the renal mesangium and segmentally along the capillary walls) which were compatable with systemic lupus erythematosus. The chest X-ray revealed patchy mottled densities in whole lung field when she complained more dyspnea at 9th hospital days. Even with the parenteral administration of broad-spectrum antibiotics, the symptoms of high fever, cough, tachydyspnea and hypoxia were continued. At 24th hospital day, the clinical course was rapidly deteriorated after sudden loss of consciousness with focal seizure which suggested CNS involvement during hydrocortisone administration for 10 days. She died of respiratory failure despite the mechanical ventilatory support with PEEP. The limited necropsy showed interstitial pneumonia, alveolar hemorrhage and occlusive necrotizing vasculitis of acute lupus pneumonitis.

• Childhood Kidney Diseases
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• v.10 no.1
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• pp.40-44
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• 2006

[ $Henoch-Sch{\"{o}}nlein$ ] purpura(HSP) is a systemic small vessel vasculitis characterized by cutaneous purpura, arthritis, abdominal pain and nephritis. The characteristic rash of HSP consists of palpable purpura on the buttocks and lower extremities. Bullous lesions often appear in adults with HSP, whereas they are very rare in children with HSP. In this case report, the patient presented with arthralgia and abdominal pain and had hemorrhagic bullae as a prominent manifestation of the disease. The skin biopsy of the patient revealed typical leukocytoclastic vasculitis of dermal vessels and prominent IgA and fibrinogen deposits on capillary walls by direct immunofluorescence. We confirmed the diagnosis of HSP and observed improvement of clinical symptoms and signs within a few days after corticosteroid treatment. We therefore report a case with a review of the literature.

• Parasites, Hosts and Diseases
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• v.60 no.2
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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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• v.25 no.1
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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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• v.4 no.1
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• pp.140-149
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• 1997

Purpose : Kawasaki Disease(KD) is a febrile disease with acute multisystemic vasculitis which is seen commonly in early childhood. The cause and etiologic agents are still unknown to identify using ordinary bacterial and viral culture, but the clinical labaratory studies suggest that KD is one of autoimmune disorder caused by infectious agents, but that is not proved yet. The study was performed to investigate the IgG subclasses in acute stage of KB before treatment of IVIG(Intravenous immunoglobulin). Method : The 35 cases in acute stage of KD before treatment of IVIG who were hospitalized from jan. 1995 to dec. 1996. The obtained sera were measured level of total IgG, IgM, IgA, IgE and IgG subclasses IgGl, IgG2, IgG3, IgG4 by using EIA and SRID method. Results : 1) The sex ratio is male to female is 1.5: 1.0, and male is prone to infected. 2) Total IgG, IgM, IgA and IgE level is normal range with age adjusted, but few cases are shown slight high level of total IgG compare to normal range of age adjusted. 3) acute phage reactants such as CRP, C3, ESR are all increased in acute stage of cases. 4) IgG subclasses IgGl, IgG2, IgG3 are shown normal range of age adjusted, but remarkably low level of IgG4 in all of cases. Conclusions : The low level of IgG4 is able to increasing the incidence of KD, and may use early diagnostic tools combine with other clinical symptoms and signs. But the resulsts of reported studies are different to each other, so it needs more times and cases to get final evaluation of changes of serum immnunoglobulin levels correlate to increase the incidence of high risk group of KD patients.

• Tuberculosis and Respiratory Diseases
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• v.67 no.3
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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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• v.63 no.6
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• pp.531-536
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• 2007

Wegener's granulomatosis is a systemic vasculitis of the medium and small arteries, as well as of the venules, arterioles, and occasionally large arteries, and primarily involves the upper and lower respiratory tracts and the kidneys. Renal symptoms of Wegener's granulomatosis are indistinguishable from those of vasculitis such as Henoch-$Sch\ddot{o}nlein$ purpura and microscopic polyangiitis. This case, though initially diagnosed as Henoch-$Sch\ddot{o}nlein$ purpura, was confirmed as Wegener's granulomatosis from a lung biopsy fifteen years after the initial diagnosis. We report this case with a review of the literature.

• Tuberculosis and Respiratory Diseases
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• v.67 no.3
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• pp.226-228
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• 2009

Henoch-$Sch\ddot{o}nlein$ purpura (HSP) is an immunologically mediated systemic vasculitis of small blood vessels that primarily involves the skin, gastrointestinal tract, joints and kidneys. HSP is a common vasculitic syndrome in children who, in most cases, achieve complete recovery. Pulmonary hemorrhage is a very rare manifestation of HSP. The authors present a case of a 46-year-old male presenting with pulmonary hemorrhage and renal involvement and the diagnosis of HSP. The patient responded to prednisolone therapy.