Fibrillary/Immunotactoid Glomerulopathy With Cardiac Involvement
A 44-year-old woman with end-stage renal disease due to fibrillary/immunotactoid glomerulopathy presented with progressive symptoms of heart failure. An echocardiogram showed a dilated left ventricle with diffuse hypokinesis. An endomyocardial biopsy revealed patchy fibrosis. Immunofluorescence demonstrated interstitial staining with immunoglobulin G and complement component C3. Electron microscopy (Figure 1) revealed deposition of fibrils with diameters ranging from 8.0 to 12.4 nm that were negative on Congo red staining and were similar to the fibrils noted on her kidney biopsy 5 years earlier (Figure 2). After aggressive fluid removal and control of her hypertension, the patient’s symptoms resolved.
This case suggests that fibrillary/immunotactoid glomerulopathy may be a systemic disease process, such as the monoclonal gammopathies or cryoglobulinemia. Immune complexes with strong intermolecular attraction may be in concentrations too low to be measured in the serum but may become concentrated in different parts of the body. Clinicians should add cardiac involvement to the expanding list of extrarenal manifestations of fibrillary/immunotactoid glomerulopathy.
Dr Sabatine is the recipient of the William A. Schreyer Clinical Fellowship in Cardiology.
Presented in part at the Laennec Society Young Clinician Competition, 73rd Scientific Sessions of the American Heart Association, New Orleans, La, November 12–15, 2000.
The editor of Images in Cardiovascular Medicine is Hugh A. McAllister, Jr, MD, Chief, Department of Pathology, St Luke’s Episcopal Hospital and Texas Heart Institute, and Clinical Professor of Pathology, University of Texas Medical School and Baylor College of Medicine.
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