Atypical antiglomerular basement membranes disease with nephrotic-range proteinuria, mesangial proliferation, and membranoproliferative glomerulonephritis pattern of injury
Joint Authors
al-Awdah, Nurah
al-Sad, Khalid O.
al-Suwaylimi, Banan
al-Suwaylimi, Ghadah
al-Hasan, al-Walid Ali
al-Sayyari, Abd Allah A.
Source
Saudi Journal of Kidney Diseases and Transplantation
Issue
Vol. 28, Issue 6 (31 Dec. 2017), pp.1397-1403, 7 p.
Publisher
Saudi Center for Organ Transplantation
Publication Date
2017-12-31
Country of Publication
Saudi Arabia
No. of Pages
7
Main Subjects
Topics
Abstract EN
Antiglomerular basement membrane (anti-GBM) disease is an uncommon autoimmune disease characterized by the presence of IgG autoantibodies targeting the alpha-3 chain of type IV collagen.
Some of the atypical forms of the disease have been described.
Herein, we describe a case of atypical anti-GBM in a 27-year-old Saudi male who presented with lower limb edema, gross hematuria, elevated serum creatinine concentration, and nephrotic-range proteinuria.
All serology tests were negative, except for anti-GBM which was weakly positive.
Renal biopsy showed proliferative glomerulonephritis (GN) with nodular transformation of the glomerular tufts, mesangial hypercellularity (mesangial cell proliferation), segmental endocapillary hypercellularity and three incomplete cellular crescents, and recapitulating membranoproliferative GN pattern of glomerular injury.
Direct immunofluorescence microscopy demonstrated diffuse, intense linear positivity for IgG and Kappa and Lambda light chains, and compatible with anti- GBM disease.
The patient was treated with cyclophosphamide and corticosteroids in addition to therapeutic plasma exchange which resulted in mild improvement in renal function over a period of six weeks.
We emphasize the importance of recognition of atypical pathological and serological patterns of anti-GBM disease, which is crucial for proper and early diagnosis and possibly improved clinical outcome and we highlight the importance of clinicopathological correlation in cases with atypical clinical and pathological presentations
American Psychological Association (APA)
al-Suwaylimi, Banan& al-Suwaylimi, Ghadah& al-Awdah, Nurah& al-Sad, Khalid O.& al-Hasan, al-Walid Ali& al-Sayyari, Abd Allah A.. 2017. Atypical antiglomerular basement membranes disease with nephrotic-range proteinuria, mesangial proliferation, and membranoproliferative glomerulonephritis pattern of injury. Saudi Journal of Kidney Diseases and Transplantation،Vol. 28, no. 6, pp.1397-1403.
https://search.emarefa.net/detail/BIM-785689
Modern Language Association (MLA)
al-Suwaylimi, Banan…[et al.]. Atypical antiglomerular basement membranes disease with nephrotic-range proteinuria, mesangial proliferation, and membranoproliferative glomerulonephritis pattern of injury. Saudi Journal of Kidney Diseases and Transplantation Vol. 28, no. 6 (Nov. / Dec. 2017), pp.1397-1403.
https://search.emarefa.net/detail/BIM-785689
American Medical Association (AMA)
al-Suwaylimi, Banan& al-Suwaylimi, Ghadah& al-Awdah, Nurah& al-Sad, Khalid O.& al-Hasan, al-Walid Ali& al-Sayyari, Abd Allah A.. Atypical antiglomerular basement membranes disease with nephrotic-range proteinuria, mesangial proliferation, and membranoproliferative glomerulonephritis pattern of injury. Saudi Journal of Kidney Diseases and Transplantation. 2017. Vol. 28, no. 6, pp.1397-1403.
https://search.emarefa.net/detail/BIM-785689
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references : p. 1402-1403
Record ID
BIM-785689