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Collapsing glomerulopathy in 19 patients with systemic lupus erythematosus or lupus-like disease.

Abstract

BACKGROUND AND OBJECTIVES

Collapsing glomerulopathy is a podocytopathy with segmental or global wrinkling and collapse of capillary walls and overlying epithelial cell proliferation. Idiopathic collapsing glomerulopathy is a distinct clinicopathologic entity with significant proteinuria, poor response to immunosuppressive therapy, and rapid progression to renal failure. Collapsing glomerulopathy is associated with viral infections, autoimmune disease, and drugs. This work presents the largest group of collapsing glomerulopathy in patients with SLE.

DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS

Clinicopathological features were retrospectively studied in 19 patients with SLE (16 patients) or SLE-like (3 patients) disease with collapsing glomerulopathy.

RESULTS

Initially, 95% of patients had nephrotic syndrome with proteinuria of 3-12 g per 24 hours, creatinine levels of 0.6-9.6 mg/dl, positive lupus serologies, and normal complement levels in 63%. Segmental and/or global collapsing glomerulopathy was seen in 11%-77% of glomeruli. Tubular atrophy with focal microcystic changes and interstitial fibrosis was seen in 35% of patients. Minimal glomerular mesangial deposits were noted in 63% of patients, and extensive foot process effacement was seen in 82% of patients. Initial treatment was with pulse/oral steroids. Follow-up from 13 patients revealed that 7 patients progressed to ESRD at the time of biopsy up to 21 months later, 1 patient returned to normal creatinine (1.1 mg/dl) without proteinuria, and 5 patients had creatinine of 1.2-3.6 mg/dl with proteinuria of 0.37-4 g per 24 hours.

CONCLUSIONS

Collapsing glomerulopathy may be seen in SLE patients presenting with massive proteinuria with or without lupus nephritis, which may have prognostic significance.

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  • Authors+Show Affiliations

    ,

    Department of Pathology, Weill Cornell Medical College, New York, New York, USA.

    , , , ,

    Source

    MeSH

    Administration, Oral
    Adolescent
    Adult
    Aged
    Atrophy
    Biopsy
    Disease Progression
    Female
    Fibrosis
    Humans
    Immunohistochemistry
    Kidney Failure, Chronic
    Kidney Glomerulus
    Kidney Tubules
    Lupus Erythematosus, Systemic
    Lupus Nephritis
    Male
    Middle Aged
    Nephrotic Syndrome
    Proteinuria
    Pulse Therapy, Drug
    Retrospective Studies
    Steroids
    Time Factors
    Treatment Outcome
    Young Adult

    Pub Type(s)

    Journal Article

    Language

    eng

    PubMed ID

    22461531

    Citation

    Salvatore, Steven P., et al. "Collapsing Glomerulopathy in 19 Patients With Systemic Lupus Erythematosus or Lupus-like Disease." Clinical Journal of the American Society of Nephrology : CJASN, vol. 7, no. 6, 2012, pp. 914-25.
    Salvatore SP, Barisoni LM, Herzenberg AM, et al. Collapsing glomerulopathy in 19 patients with systemic lupus erythematosus or lupus-like disease. Clin J Am Soc Nephrol. 2012;7(6):914-25.
    Salvatore, S. P., Barisoni, L. M., Herzenberg, A. M., Chander, P. N., Nickeleit, V., & Seshan, S. V. (2012). Collapsing glomerulopathy in 19 patients with systemic lupus erythematosus or lupus-like disease. Clinical Journal of the American Society of Nephrology : CJASN, 7(6), pp. 914-25. doi:10.2215/CJN.11751111.
    Salvatore SP, et al. Collapsing Glomerulopathy in 19 Patients With Systemic Lupus Erythematosus or Lupus-like Disease. Clin J Am Soc Nephrol. 2012;7(6):914-25. PubMed PMID: 22461531.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - Collapsing glomerulopathy in 19 patients with systemic lupus erythematosus or lupus-like disease. AU - Salvatore,Steven P, AU - Barisoni,Laura M C, AU - Herzenberg,Andrew M, AU - Chander,Praveen N, AU - Nickeleit,Volker, AU - Seshan,Surya V, Y1 - 2012/03/29/ PY - 2012/3/31/entrez PY - 2012/3/31/pubmed PY - 2012/10/17/medline SP - 914 EP - 25 JF - Clinical journal of the American Society of Nephrology : CJASN JO - Clin J Am Soc Nephrol VL - 7 IS - 6 N2 - BACKGROUND AND OBJECTIVES: Collapsing glomerulopathy is a podocytopathy with segmental or global wrinkling and collapse of capillary walls and overlying epithelial cell proliferation. Idiopathic collapsing glomerulopathy is a distinct clinicopathologic entity with significant proteinuria, poor response to immunosuppressive therapy, and rapid progression to renal failure. Collapsing glomerulopathy is associated with viral infections, autoimmune disease, and drugs. This work presents the largest group of collapsing glomerulopathy in patients with SLE. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Clinicopathological features were retrospectively studied in 19 patients with SLE (16 patients) or SLE-like (3 patients) disease with collapsing glomerulopathy. RESULTS: Initially, 95% of patients had nephrotic syndrome with proteinuria of 3-12 g per 24 hours, creatinine levels of 0.6-9.6 mg/dl, positive lupus serologies, and normal complement levels in 63%. Segmental and/or global collapsing glomerulopathy was seen in 11%-77% of glomeruli. Tubular atrophy with focal microcystic changes and interstitial fibrosis was seen in 35% of patients. Minimal glomerular mesangial deposits were noted in 63% of patients, and extensive foot process effacement was seen in 82% of patients. Initial treatment was with pulse/oral steroids. Follow-up from 13 patients revealed that 7 patients progressed to ESRD at the time of biopsy up to 21 months later, 1 patient returned to normal creatinine (1.1 mg/dl) without proteinuria, and 5 patients had creatinine of 1.2-3.6 mg/dl with proteinuria of 0.37-4 g per 24 hours. CONCLUSIONS: Collapsing glomerulopathy may be seen in SLE patients presenting with massive proteinuria with or without lupus nephritis, which may have prognostic significance. SN - 1555-905X UR - https://www.unboundmedicine.com/medline/citation/22461531/Collapsing_glomerulopathy_in_19_patients_with_systemic_lupus_erythematosus_or_lupus_like_disease_ L2 - http://cjasn.asnjournals.org/cgi/pmidlookup?view=long&pmid=22461531 DB - PRIME DP - Unbound Medicine ER -