Abstract
Anti-glomerular basement membrane (anti-GBM) disease occurs in fewer than two cases per million population. Patients usually present with features of rapidly progressive glomerulonephritis (RPGN) with or without pulmonary involvement. Anti-GBM disease is classically diagnosed by both demonstrating GBM linear immunofluorescence staining on kidney biopsy and detecting anti-GBM antibodies in serum. More than 90% of patients with anti-GBM disease either become dialysis-dependent or die if left untreated.Here, we report a 37-year-old man who presented with bilateral lower limb edema, hypertension, acute kidney injury (creatinine of 212 μmol/L), microscopic hematuria, and nephrotic range proteinuria (15 g/day). His kidney biopsy showed diffuse crescentic membranoproliferative glomerulonephritis and bright linear staining of GBM by immunoglobulin G consistent with anti-GBM disease; however, serum anti-GBM antibodies were negative. The patient was diagnosed with atypical anti-GBM disease and treated aggressively with intravenous pulse steroids, plasmapheresis, oral cyclophosphamide, and oral prednisolone with significant improvement in kidney function and proteinuria.Atypical anti-GBM disease should be considered in patients presenting with RPGN, even in the absence of serum anti-GBM antibodies. Early diagnosis and aggressive treatment in such cases are warranted to prevent irreversible kidney damage as the course of the disease might not be as benign as previously thought.
Highlights
Anti-glomerular basement membrane disease is a rare autoimmune disorder that often presents as rapidly progressive glomerulonephritis (RPGN), with or without pulmonary manifestations.[1]
Patients usually present with features of rapidly progressive glomerulonephritis (RPGN) with or without pulmonary involvement
Atypical anti-GBM disease is a rare variant characterized by diffuse linear staining of GBM by immunoglobulin G (IgG) on immunofluorescence microscopy and absence of circulating serum anti-GBM antibodies by enzyme-linked immunosorbent assay (ELISA), Western blot, or indirect immunofluorescence.[5]
Summary
Anti-glomerular basement membrane (anti-GBM) disease is a rare autoimmune disorder that often presents as rapidly progressive glomerulonephritis (RPGN), with or without pulmonary manifestations.[1]. Mr S, a 37-year-old Sri Lankan man with no significant past medical history of any chronic illness, presented to the emergency department in December 2016 with progressive bilateral lower limb swelling for 10 days. He reported subjective fever, cough, and dark urine that he developed a few days before hospital presentation. The patient denied chest pain, shortness of breath, orthopnea, hemoptysis, nausea, vomiting, abdominal pain, diarrhea, joint pain, skin rash, or dysuria His physical examination was notable for a blood pressure of 197/110 mmHg and bilateral pitting lower limb edema without other significant findings. All viral and immunologic workups were negative, including anti-GBM antibodies (two samples),
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