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Table 2 Key publications regarding the efficacy of immunosuppressive drugs in treating fibrillary glomerulonephritis

From: Fibrillary glomerulonephritis with small fibrils in a patient with the antiphospholipid antibody syndrome successfully treated with immunosuppressive therapy

Treatment

Response

Authors

Methyprednisolone/Prednisolone

No response

Asaba et al., 2003

Prednisolone & Mycophenolate mofetil

Persistent haematuria and proteinuria, improvement in serum creatinine levels

Bijol et al., 2006

Prednisolone, Cyclophosphamide then Cyclosporin

Brief initial response with prednisolone then relapse. No response with cyclophosphamide resolution of nephrotic syndrome with cyclosporin

Bircan et al., 2004

Prednisolone & Cyclophosphamide

Marked improvement in renal function

Blume et al., 2002

Corticosteroids +/- Cyclophosphamide

No response in fibrillary glomerulonephritis.

Variable response in immunotactoid glomerulopathy including melphalan, vincristine, doxorubicin, carmustine or chlorambucil in various combinations, remission or improvement in nephrotic syndrome in 10/12 patients, disappearance of deposits from the kidneys of 2 patients

Bridoux et al., 2002

Prednisolone & Cyclophosphamide then Azathioprine

Transient reduction in proteinuria

Chan han, 1998

Prednisolone

Resolution of nephrotic syndrome or reduction in proteinuria

Dickenmann et al., 2002

Prednisolone & Chlorambucil

Slight reduction in creatinine, reduction in proteinuria from 3 g to 1 g in 24 h.

Dussol et al., 1998

Prednisolone

No response

Gielen et al., 2006

Prednisolone & Cyclophosphamide then Azathioprine

No response

Hsu et al., 2001

Prednisolone & Cyclophosphamide

Recovery of renal function, discontinuation of dialysis

Mahajan et al., 2005

Prednisolone

Improvement in renal function, reduction in proteinuria

Moriyama et al., 2003

Prednisolone & Chloraminophen (Chlorambucil)

Reduction in creatinine levels, disappearance of proteinuria and haematuria

Nabarra et al., 2003

Prednisolone & Mycophenolate mofetil

Continued rapid deterioration in renal function

Ovuworie et al., 2000

Prednisolone, Cyclophosphamide, plasmapheresis & immunoglobulins

Fibrils in renal transplant patient, continued rapid deterioration in renal function

Palanichamy et al., 1998

Various combinations of Cyclosporin A

Biopsy proven recurrence of fibrillary glomerulopathy in 3 transplanted kidneys

Pronovost et al., 1996

Prednisolone, Azathioprine and ATG (in 1 patient)

Rate of decline in renal allografts slower than native kidneys suggesting either benefit of immunosuppressive therapy or spontaneous remission with time

 

20 treated patients out of 56

No effect of immunosuppression on incidence of or time to end stage renal disease

Rosenstock et al., 2003

Steroids alone (16% of patients)

Variable response including improvement in renal function and reduction in proteinuria

 

Cyclophosphamide +/- steroids (14%)

Cyclosporin (5%)

Poor response of patients with immunotactoid glomerulopathy to immunosuppression except one good responder to fludarabine with improvement in renal function and reduced proteinuria

 

Prednisolone & Cyclophosphamide

Recovery of renal function, discontinuation of dialysis, recovery from pulmonary haemorrhage

Rovin et al., 1993

Prednisolone & Chlorambucil

Reduction or stabilization in creatinine levels, reduction in proteinuria

Schneider et al., 1996