Collapsing glomerulopathy in a patient with mixed connective tissue disease

Rifkin, Stephen I.; Gutta, Hemalatha; Nair, Ramesh; McFarren, Christopher; Wheeler, Donald E.

doi:10.5414/cn106425

Cited by 3 publications

(2 citation statements)

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“…Knowledge about TMA-associated CG (TMA-CG) is scarce and mostly based on sporadic reports of CG occurring in renal transplant recipients, in association with immunosuppressive drugs' toxicity. [10][11][12] In native kidneys, TMA-CG has only been anecdotally described in case reports or small series in conjunction with calcineurin inhibitors-related arteriolopathy, 13 sickle-cell disease, 14 antivascular endothelial growth factor (anti-VEGF) therapy-associated TMA, 15 mixed connective tissue disease-associated TMA, 16 or malignant hypertension. 17 The question of the prevalence and significance of CG changes in the setting of histological TMA has never been addressed in an unselected fashion, using native renal biopsy specimens.…”

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Collapsing glomerulopathy is common in the setting of thrombotic microangiopathy of the native kidney

Buob

Decambron

Gnemmi

et al. 2016

Kidney International

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Thrombotic microangiopathy (TMA) is a poorly recognized cause of collapsing glomerulopathy. The frequency and significance of collapsing glomerulopathy associated with renal TMA have not been specifically studied in native kidney biopsy specimens. Here we retrospectively documented clinicopathologic features of 53 patients with histologically proven TMA in the native kidney, with special emphasis on changes due to focal segmental glomerulosclerosis (FSGS). Histological TMA was related to hypertensive nephropathy in 21 patients, genetic complement abnormalities in 9, drugs in 7, and to other causes in 16 patients. Almost half (26 patients) presented with arteriolar, 6 with glomerular, and 21 with mixed TMA. Using the Columbia classification system for the 53 patients with histological TMA, 33 had concurrent FSGS lesions with collapsing glomerulopathy the dominant variant in 19 patients (58% of the FSGS cases), not otherwise specified in 9 patients, cellular in 3, and perihilar or tip lesions in 1 patient each. The presence of FSGS was associated with a poor renal prognosis, with no prognostic difference between collapsing glomerulopathy and other FSGS variants. Thus, collapsing glomerulopathy is frequently found in native kidney biopsies with TMA, suggesting that endothelial injury may play an important role in the pathophysiology of FSGS.

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confidence: 99%

Collapsing glomerulopathy is common in the setting of thrombotic microangiopathy of the native kidney

Buob

Decambron

Gnemmi

et al. 2016

Kidney International

View full text Add to dashboard Cite

show abstract

“…3 CG has been described in association with a variety of infections, malignancies, genetic diseases, drugs and post-transplantation. It has been associated with several autoimmune diseases, including systemic lupus erythematosus (SLE) 4 and Sjo¨gren's disease, 5 as well as rare cases of mixed connective tissue disease (MCTD), 6 adult Still's disease 7 and cerebral arteritis 5 (Table 1). In the renal literature, CG has been reported in patients with collagen vascular-like disease who have marked serological abnormalities but minimal clinical findings that do not meet criteria for definite collagen vascular disease.…”

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confidence: 99%