A Case of Fibrillary Glomerulonephritis Associated with Thrombotic Microangiopathy and Anti-Glomerular Basement Membrane Antibody

Abstract

We present the first report of a case of fibrillary glomerulonephritis (FGN) associated with thrombotic microangiopathy (TMA) and anti-glomerular basement membrane antibody (anti-GBM antibody). A 54-year-old man was admitted to our hospital for high fever and anuria. On the first hospital day, we initiated hemodialysis for renal dysfunction. Laboratory data revealed normocytic-normochromic anemia with schistocytes in the peripheral smear, thrombocytopenia, increased serum lactate dehydrogenase, decreased serum haptoglobin, and negative results for both direct and indirect Coombs tests. Based on these results, we diagnosed TMA. Assays conducted several days later indicated a disintegrin-like and metalloprotease with a thrombospondin motif 13 (ADAMTS13) activity of 31.6%, and ADAMTS13 inhibitors were negative. We started plasma exchange using fresh frozen plasma and steroid pulse therapy. Anti-GBM antibody was found to be positive. Renal biopsy showed FGN. Blood pressure rose on the 46th hospital day, and mild convulsions developed. Based on magnetic resonance imaging of the head, the patient was diagnosed with reversible posterior leukoencephalopathy syndrome. Hypertension persisted despite administration of multiple antihypertensive agents, and the patient experienced a sudden generalized seizure. Computed tomography of the head showed multiple cerebral hemorrhages. However, his blood pressure subsequently decreased and the platelet count increased. TMA remitted following 36 plasma exchange sessions, but renal function was not restored, and maintenance hemodialysis was continued. The patient was discharged on the 119th day of hospitalization. In conclusion, it was shown that TMA, FGN and anti-GBM antibody were closely related.

Rapidly progressive glomerulonephritis due to coexistent anti-glomerular basement membrane disease and fibrillary glomerulonephritis

“…In contrast to the well-known finding of positivity of ANCA, especially anti-myeloperoxidase-ANCA, in 30–38% of patients with anti-GBM disease [ 23 , 24 ], the presence of circulating anti-GBM IgG antibody in patients with FGN has been demonstrated only in one other recent case [ 18 ] (Table 1 ). Momose et al [ 18 ] described a patient who presented with acute kidney injury and positive serum anti-GBM antibody; renal biopsy showed thrombotic microangiopathy and ultrastructural features of FGN. Proliferative GN pattern and global sclerosis were described on light microscopy of 17 glomeruli.…”

“… Abundant, fairly straight, nonbranching fibrils with mean diameter of 26 nm in the thickened GBM and expanded mesangial regions. Reported cases of FGN presenting as crescentic GN with positive anti-GBM antibody 1 [ 18 ] 54 Male Positive Proliferative GN pattern and global sclerosis were described on light microscopy of 17 glomeruli. Renal arterioles, both afferent and efferent, showed extensive endothelial cell edema and swelling.…”

“…Although there are no controlled trials to guide treatment for FGN, high-dose glucocorticoids and cyclophosphamide have been used as treatment for FGN with crescentic GN [ 5 , 9 ]. Its prognosis, however, is poor, and all reported cases of FGN with the clinical presentation of RPGN required renal replacement therapy and remained dialysis dependent [ 5 , 8 , 9 , 18 ]. Plasmapheresis in conjunction with prednisone and cyclophosphamide have been recommended for the treatment of anti-GBM disease except in patients who are dialysis dependent at presentation with 100% crescents in kidney biopsy (correlated with poor kidney survival) and do not have pulmonary hemorrhage [ 33 ].…”

Clinical characteristics of anti-GBM disease with thrombotic microangiopathy: a case report and literature review

“…Several cases of anti-GBM disease with TMA have been reported [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20]; however, the clinical characteristics of this phenomenon remain unclear. Therefore, we conducted a literature review on this phenomenon (Table 2).…”

“…Autoimmune diseases, such as systemic lupus erythematosus, systemic sclerosis, antiphospholipid syndrome, and anti-neutrophil cytoplasmic autoantibody (ANCA)associated vasculitis, have been reported to be the cause of secondary TMA [2,3,5]. Though there are some existing case reports of anti-GBM disease with TMA [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20], the clinical features of this phenomenon have not been described extensively.…”