Rapidly progressive ('crescentic') glomerulonephritis and monoclonal gammapathies

Area of Science:

• Nephrology
• Hematology
• Oncology

Background:

• Rapidly progressive glomerulonephritis (RPGN) is a severe kidney disease characterized by rapid loss of kidney function.
• Plasma cell dyscrasias, including Waldenstrom's macroglobulinemia, are conditions involving abnormal plasma cells that can produce monoclonal proteins.
• The association between monoclonal gammopathies and glomerulonephritis, particularly crescentic glomerulonephritis, is increasingly recognized.

Observation:

• Three patients with RPGN were diagnosed with plasma cell dyscrasia (2 cases) or Waldenstrom's macroglobulinemia (1 case).
• Renal biopsies revealed proliferative glomerulonephritis with significant endo- and extracapillary (crescentic) proliferation in all three patients.
• Literature review identified 13 additional cases of proliferative glomerulonephritis associated with monoclonal gammopathies, including 3 with crescents.

Findings:

• Treatment outcomes varied: one patient improved with corticosteroids and cyclophosphamide, another progressed to hemodialysis without treatment, and a third improved with plasma exchange and cytotoxic drugs, avoiding dialysis.
• These findings suggest that B-cell dyscrasias are a potential cause of crescentic glomerulonephritis.
• Successful treatment of the hematologic disorder led to dramatic improvement in renal function in one case.

Implications:

• B-cell dyscrasias should be considered in the differential diagnosis of patients presenting with crescentic glomerulonephritis.
• Therapeutic strategies targeting the underlying hematologic malignancy may offer a significant opportunity for renal recovery in affected individuals.
• This highlights the importance of a multidisciplinary approach involving nephrologists and hematologists for optimal patient management.