Primary Glomerular Disease

Nachman, Patrick H.; Jennette, J. Charles; Falk, Ronald J.

doi:10.1016/b978-1-4160-6193-9.10031-4

Cited by 60 publications

(80 citation statements)

References 1,030 publications

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“…These findings suggest that MCD cannot explain all cases of IgAN with NS. As suggested previously, nephrotic range proteinuria in patients with IgAN might be caused by other forms of NS or accompanying GN (6,14).…”

Section: Discussionmentioning

confidence: 52%

“…Baseline characteristics of the 985 patients who met the inclusion criteria are shown in Table 1. Among these patients, NS occurred in 100 (10.2%), with 93 having generalized edema as the first presenting symptom and undergoing renal biopsy within an average of 7 days of symptom onset (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17). The remaining seven patients initially had microscopic hematuria with minimal proteinuria (UPCR # 0.5 g/g) but presented with acute NS onset during follow-up.…”

Section: Baseline Characteristicsmentioning

confidence: 99%

“…Fewer than 10% of patients present with uncommon features such as ARF or nephrotic syndrome (NS) (5,6). In particular, NS occurs in only 5% of all patients with IgAN (2).…”

Section: Introductionmentioning

confidence: 99%

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Clinical Features and Outcomes of IgA Nephropathy with Nephrotic Syndrome

Kim¹,

Kim²,

Lee

et al. 2012

Clinical Journal of the American Society of Nephrology

100

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Summary Background and objectives Nephrotic syndrome (NS) is a rare manifestation of IgA nephropathy (IgAN). Clinical characteristics and long-term outcomes of this condition have not yet been explored. Design, setting, participants, & measurements A multicenter observational study was conducted between January 2000 and September 2010 in 1076 patients with biopsy-proven IgAN from four medical centers in Korea. The primary outcome was a doubling of the baseline serum creatinine concentration. Results Of the 1076 patients, 100 (10.2%) presented with NS; complete remission (CR), partial remission (PR), and no response (NR) occurred in 48 (48%), 32 (32%), and 20 (20%) patients, respectively. During the median follow-up of 45.2 months, 24 patients (24%) in the NS group reached the primary endpoint compared with 63 (7.1%) in the non-NS group (P<0.001). The risk of reaching the primary endpoint was significantly higher in the PR (P=0.04) and NR groups (P<0.001) than in the CR group. Among patients with NS, 24 (24%) underwent spontaneous remission (SR). SR occurred more frequently in female patients and in patients with serum creatinine levels ≤1.2 mg/dl and a >50% decrease in proteinuria within 3 months after NS onset. None of the patients with SR reached the primary endpoint and they had fewer relapses during follow-up. Conclusions This study demonstrated that the prognosis of NS in IgAN was not favorable unless PR or CR was achieved. In addition, SR was more common than expected, particularly in patients with preserved kidney function and spontaneous decrease in proteinuria shortly after NS onset.

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Section: Discussionmentioning

confidence: 52%

Section: Baseline Characteristicsmentioning

confidence: 99%

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Clinical Features and Outcomes of IgA Nephropathy with Nephrotic Syndrome

Kim¹,

Kim²,

Lee

et al. 2012

Clinical Journal of the American Society of Nephrology

100

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“…ANCA-positive MPA should be considered as part of the spectrum of ANCA-associated small-vessel vasculitis, ranging from renal-limited rapidly progressive glomerulonephritis (RPGN) to multisystem diseases, including Wegener's granulomatosis and Churg-Strauss syndrome [5,6]. Corticosteroids are frequently used in the treatment of ANCA-positive RPGN.…”

Section: Discussionmentioning

confidence: 99%

De novo acute hepatitis B in myeloperoxidase-specific anti-neutrophil cytoplasmic autoantibody-related microscopic polyangiitis treated with corticosteroids

Iwafuchi¹,

Oyama²,

Morita

et al. 2012

CEN Case Rep

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An 82-year-old female was referred to our hospital because of low-grade fever, anemia, and rapidly progressive nephritic syndrome. Her laboratory data showed mild proteinuria, mild renal failure, and the presence of myeloperoxidase-specific anti-neutrophil cytoplasmic autoantibody. A skin biopsy specimen taken from the erythematous purpura revealed neutrophilic infiltration around the blood vessels with fibrinoid changes in the vessel walls. A renal biopsy specimen revealed segmental necrotizing glomerulonephritis with fibro-cellular crescent formation without deposits of immunoglobulin or complement components, indicating microscopic polyangiitis. The use of corticosteroid treatment, including intravenous methylprednisolone, improved renal failure. After 4 years with low-dose maintenance corticosteroid therapy, she developed de novo acute hepatitis B, and entecavir was remarkably effective, showing a rapid recovery from liver dysfunction with jaundice. To prevent hepatitis B virus (HBV) reactivation and de novo acute hepatitis B induced by immunosuppressive or cytotoxic therapy, including corticosteroids alone, the measurement of HBV-related serological markers needs to be performed prior to the initiation of such therapy, even in renal diseases.

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“…In idiopathic MPGN, low serum levels of C3 and CH50 were observed, whereas the C4 level tends to be normal [18]. When MPGN is caused by cryoglobulinemia, however, C4 depression is more prominent than C3 [19]. This case should be diagnosed as secondary MPGN, if not due to cryoglobulinemia, in which the classical complement pathway is involved.…”

Section: Discussionmentioning

confidence: 99%

A case of membranoproliferative glomerulonephritis developed over twenty years with three different findings of renal pathology

et al. 2012

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A 31-year-old woman with proteinuria, hypocomplementemia, rheumatoid factor, and high serum polyclonal IgM concentration was admitted to our hospital for renal biopsy. She had a past history of two renal biopsies. When she was 12 years old, she developed proteinuria, microscopic hematuria, and hypocomplementemia. She was diagnosed as having 'IgM nephropathy' based on minor glomerular abnormalities as determined by light microscopy and IgM and C3 deposition in the mesangial region by immunofluorescence microscopy at the first biopsy. Despite corticosteroid treatment, her proteinuria did not improve and she discontinued regular outpatient checkups. When she was 29 years old and pregnant, she developed preeclampsia and, after delivery, a second renal biopsy was implemented. She was diagnosed as having progressed 'IgM nephropathy' with endotheliosis induced by preeclampsia. She was treated with angiotensin II receptor blocker and her proteinuria diminished; however, 1 year after the delivery, she developed proteinuria again, along with microscopic hematuria and hypocomplementemia. A third renal biopsy was conducted at 31 years of age and she was diagnosed as having membranoproliferative glomerulonephritis (MPGN) type I on the basis of diffuse mesangial proliferation, endocapillary hypercellularity with double contour of the capillary wall, and lobular formation in glomeruli, as determined by light microscopy. Immunofluorescence staining demonstrated deposits of C3, C4, C1q, and IgM in the mesangial region and capillary wall. She underwent corticosteroid therapy followed by normalization of urinalysis and serum complement level. Although she had initially been diagnosed with 'IgM nephropathy', she was finally diagnosed with secondary MPGN and was successfully treated by corticosteroid therapy.

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Primary Glomerular Disease

Cited by 60 publications

References 1,030 publications

Clinical Features and Outcomes of IgA Nephropathy with Nephrotic Syndrome

Clinical Features and Outcomes of IgA Nephropathy with Nephrotic Syndrome

De novo acute hepatitis B in myeloperoxidase-specific anti-neutrophil cytoplasmic autoantibody-related microscopic polyangiitis treated with corticosteroids

A case of membranoproliferative glomerulonephritis developed over twenty years with three different findings of renal pathology

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