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Diagnosis of monoclonal immunotactoid glomerulopathy with positive λ chain by immunoelectron microscopy
Erina Sugita1, Homare Sonoda1, Masaki Ryuzaki1
1Division of Endocrinology, Metabolism and Nephrology, Department of Internal Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan.
Insights
Diagnosing monoclonal immunotactoid glomerulopathy (ITG) can be challenging. Immunoelectron microscopy and serum/urine protein electrophoresis are crucial for detecting monoclonality when routine tests are inconclusive.
Area of Science:
- Nephrology
- Pathology
- Immunology
Background:
- Immunotactoid glomerulopathy (ITG) is a rare kidney disease.
- ITG involves glomerulonephritis and microtubular deposits in capillary walls.
- Distinguishing monoclonal ITG from polyclonal ITG is important for prognosis and treatment.
Observation:
- A 73-year-old man presented with ITG.
- Routine immunofluorescence was negative for IgG, IgA, and IgM.
- Immunoelectron microscopy revealed a positive lambda (λ) light chain.
Findings:
- Monoclonal ITG is associated with hematological disorders and better treatment response.
- Serum and urine analysis 6 months post-biopsy detected IgG lambda chain, indicating monoclonality.
- This case highlights diagnostic challenges in monoclonal ITG.
Implications:
- Immunoelectron microscopy is valuable for diagnosing ITG when routine methods fail.
- Follow-up serum and urine protein electrophoresis with immunofixation aids in detecting monoclonality.
- Early and accurate diagnosis of monoclonal ITG improves patient outcomes.
Abstract:
We report the case of a 73-year-old-man who developed immunotactoid glomerulopathy (ITG). ITG is a rare disease characterized by proliferative glomerulonephritis and capillary wall deposits with a 10-60 nm diameter microtubular substructure. In monoclonal ITG, immunofluorescence analysis typically exhibits IgG with light chain restriction. Recent reviews recommend distinguishing monoclonal ITG from polyclonal ITG because monoclonal ITG is associated with a higher incidence of hematological disorders and better responsiveness to clone-directed therapy and renal prognosis. In our case, IgG, IgA, and IgM were negative by routine immunofluorescence; however, immunoelectron microscopy revealed positive λ chain. At 6 months after renal biopsy, the IgG λ chain was detected in the serum and urine, reflecting possible monoclonality. Therefore, it is useful to perform immunoelectron microscopy and follow-up with serum and urine protein electrophoresis and immunofixation to diagnose monoclonal ITG, even when routine immunofluorescence shows negative or nonspecific findings.
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