Atypical antiglomerular basement membranes disease with nephrotic-range proteinuria, mesangial proliferation, and membranoproliferative glomerulonephritis pattern of injury

• Published in: Saudi journal of kidney diseases and transplantation Vol. 28; no. 6; pp. 1397 - 1403
• Main Authors: AlSowailmi, Banan, AlSowailmi, Ghada, Aloudah, Nourah, Alsaad, Khaled O, Elhassan, Elwaleed, Al Sayyari, Abdulla A
• Format: Journal Article
• Language: English
• Published: Riyadh, Saudi Arabia Saudi Center for Organ Transplantation 01-11-2017; Medknow Publications and Media Pvt. Ltd; Medknow Publications & Media Pvt. Ltd; Wolters Kluwer Medknow Publications
• Subjects: Autoantibodies; Biopsy; Blood; Care and treatment; Cell proliferation; Diagnosis; Dosage and administration; Edema; Glomerulonephritis; Health sciences; Immunoglobulins; Laboratories; Medicine; Membranes; Pathology; Proteins; Proteinuria; Urine; الأجسام المضادة; البروتين البولي; التشخيص; التهاب كبيبات الكلى; الغشاء القاعدي; المتلازمة الكلوية
• ISSN: 1319-2442; 2320-3838
• DOI: 10.4103/1319-2442.220868

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