Henoch-Schönlein purpura nephritis in a patient with IgG4-related disease: A possible association

Ito, Kiyoaki; Yamada, Kazunori; Mizushima, Ichiro; Aizu, Motohiko; Fujii, Hiroshi; Mizutomi, Kazuaki; Matsumura, Masami; Hayashi, Kenshi; Yamagishi, Masakazu; Umehara, Hisanori; Yamaguchi, Yutaka; Nagata, Michio; Kawano, Mitsuhiro

doi:10.5414/cn107114

Cited by 28 publications

(15 citation statements)

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“…5). 5,7,10,[12][13][14][15]30 Four of the 43 patients had been followed up for primary Sjögren's syndrome before 2004. The diagnosis of extrarenal lesions was made on the basis of physical findings and the results of imaging studies (CT and gallium citrate scintigraphy) and/or biopsy, in addition to exclusion of other diseases.…”

Section: Materials and Methods Patientsmentioning

confidence: 99%

“…Renal pathology data were available for 30 patients, and all of them were found to have characteristic IgG4-related TIN. 7 Glomerular lesions other than global sclerosis were evident in 10 of the 30 patients: Henoch-Schönlein purpura nephritis in two, 13,14 membranous glomerulonephritis in two, 10,15 focal and segmental endocapillary proliferative glomerulonephritis in two, mesangioproliferative glomerulonephritis (with mild IgG and IgA deposition in the glomeruli) in two, IgA nephropathy in one, and membranoproliferative glomerulonephritis in one patient. Four patients had a history of malignancy at the time of IgG4-RKD diagnosis (rectal cancer, breast cancer, urinary bladder cancer, and gastric cancer in one each, respectively).…”

Section: Baseline Characteristicsmentioning

confidence: 97%

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The clinical course of patients with IgG4-related kidney disease

Saeki

Kawano

Mizushima

et al. 2013

Kidney International

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Long-term follow-up for IgG4-related kidney disease, including relapse information, is sparse. To gather data on this we retrospectively examined the clinical course of 43 patients with IgG4-related kidney disease, in which most patients were treated with, and maintained on, corticosteroids. One month after the start of treatment, most of the abnormal serology and radiology parameters had improved. In 34 of the steroid-treated patients whose follow-up period was more than 12 months (median 34 months), excluding one hemodialysis patient, the estimated glomerular filtration rate (eGFR) before treatment was over 60 ml/min in 14 patients (group A) and under 60 ml/min in 20 patients (group B). In group A, there was no difference between the eGFR before therapy and at the last review. In group B, the mean eGFR before treatment (34.1 ml/min) was significantly improved after 1 month (45.0 ml/min), and renal function was maintained at a similar level through last follow-up. Among 24 evaluated patients at the last review, however, renal atrophy had developed in 2 of 9 in group A and in 9 of 15 in group B. Relapse of IgG4-related lesions occurred in 8 of 40 treated patients. Thus, the response of IgG4-related kidney disease to corticosteroids is rapid, not total, and the recovery of renal function persists for a relatively long time under low-dose maintenance. A large-scale prospective study to formulate more useful treatment strategies is necessary.

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Section: Materials and Methods Patientsmentioning

confidence: 99%

Section: Baseline Characteristicsmentioning

confidence: 97%

The clinical course of patients with IgG4-related kidney disease

Saeki

Kawano

Mizushima

et al. 2013

Kidney International

Self Cite

157

187

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“…Similarly, Henoch À Schonlein purpura nephritis concurrent with IgG4-TIN is of interest. 24,25 In IgG4-RD, allergic manifestations are often observed and IgG4 is produced in response to repeated exposure to environmental antigens. 3 In previous case reports, such an allergic predisposition or response to repeated allergen exposure had been discussed as a possible cause of both the Henoch À Schonlein purpura nephritis and IgG4-TIN.…”

Section: Glomerular Lesionsmentioning

confidence: 99%

IgG4-related kidney disease

Saeki

Kawano

2014

Kidney International

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123

126

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IgG4-related kidney disease (IgG4-RKD) is a comprehensive term for renal lesions associated with IgG4-related disease, which is a recently recognized clinical entity characterized by a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells with fibrosis affecting several organs. Tubulointerstitial nephritis with increased IgG4-positive plasma cells and fibrosis is the most dominant feature of IgG4-RKD and may cause acute or chronic renal dysfunction, although some glomerular lesions such as membranous nephropathy are sometimes evident. Radiologically, several characteristic abnormalities are often demonstrated, sometimes mimicking malignancies. IgG4-RKD predominantly affects middle-aged to elderly men, and most patients have accompanying IgG4-related extrarenal lesions such as sialadenitis, lymphadenopathy, or type 1 autoimmune pancreatitis. Serology usually demonstrates high levels of serum total IgG and IgG4, and high levels of serum IgE and hypocomplementemia are also frequent features. Corticosteroid therapy is usually quite effective, leading to amelioration of the renal dysfunction and radiological and serological abnormalities. However, as any delay in treatment may result in irreversible renal failure, early diagnosis and appropriate therapy are very important. Despite these distinctive clinicopathological features of IgG4-RKD, its pathogenesis remains poorly understood. Awareness of this condition and accumulation of more cases worldwide are necessary.

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“…The complete mechanism and pathology of IgG4‐RD remain elusive. There are several reports suggesting that the skin and systemic vasculitis lesions possibly coincide with IgG4‐RD . Collectively, these reported findings indicate the need for increased knowledge of IgG4‐RD for a better understanding of the underlying mechanisms of this disease.…”

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confidence: 78%