Rapidly Progressive (‘Crescentic’) Glomerulonephritis and Monoclonal Gammapathies

Abstract

In 3 patients rapidly progressive glomerulonephritis revealed plasma cell dyscrasia in 2 cases and Waldenstrom’s macroglobulinemia in the 3rd. Renal biopsy showed proliferative glomerulonephritis with marked endo- and extracapillary (crescentic) proliferation. In case 1, corticosteroids and cyclophosphamide favorably influenced the course of the disease. The 2nd patient was not treated and went on to maintenance hemodialysis. In the 3rd case, plasma exchange and cytotoxic drugs allowed discontinuation of hemodialysis. Thirteen other cases of proliferative glomerulonephritis associated with monoclonal gammapathies have been described in the literature, including 3 with crescents. B-cell dyscrasia should be considered a possible etiology in a patient with crescentic glomerulonephritis. In such case we show that treatment of the hematologic disorder can be followed by a dramatic improvement of renal function.

Renal Lesions Associated with IgM-Secreting Monoclonal Proliferations

“…There were 11 men and three women aged 55 to 75 yr at the time of diagnosis of kidney disease (mean age 67.6 yr). Renal symptoms included a nephrotic syndrome in seven patients (4,5,6,7,8,12, and 13) associated with acute kidney injury in four cases (6, 7, 8, and 12). The average daily proteinuria in patients with the nephrotic syndrome was 7.2 g (4.5 to 16.0 g).…”

“…At the time of renal disease diagnosis, the lymphoproliferative disorder consisted of WM in seven patients (1,2,3,7,8,9, and 11), small B cell non-Hodgkin lymphoma in two patients (4 and 12), multiple myeloma in one patient (patient 5), and B cell lymphoma of the splenic marginal zone in one patient (patient 10). Three patients (6, 13, and 14) had an "IgM-related disorder.…”

“…First reviewed by Morel-Maroger et al (3), WM-related nephropathies include characteristic intracapillary deposits of IgM with or without cryoglobulinemia, AL-amyloidosis, and infiltration of the interstitium by neoplastic lymphoplasmacytic cells. Subsequent case reports included immunotactoid and nonamyloid fibrillary glomerulopathy (4,5), cryoglobulinemia-related glomerulonephritis (6), and crescentic glomerulonephritis (7,8). Cases of cast nephropathy (9), Fanconi syndrome (10), and LC deposition disease (11) as a result of free LC toxicity have also been reported in patients who presented with a circulating monoclonal IgM.…”

Recurrence of Light Chain Nephropathy in a Renal Allograft

“…The mechanisms by which it can do this include nephrocalcinosis, renal stones, myeloma kidney, amyloidosis and the entity known as light chain nephropathy. It is clear that benign paraproteinaemias can also cause renal impairment if the light chains produced have specific features that make them directly toxic to the kidney [1,2]. The Pi of the immunoglobulin protein has been implicated in this nephrotoxic potential [3].…”

Hypocomplementemic Proliferative Glomerulonephritis with C3 Nephritic-Factor-Like Activity in Multiple Myeloma

“…A review of the literature revealed an addi tional 18 patients with dysproteinemic states (excluding essential cryoglobulinemia) where a proliferative glom erulonephritis was however the predominant histological finding [5][6][7][8][9][10][11][12][13][14][15][16][17][18]. Ten had multiple myeloma [5][6][7][8][9]12,15,17,18], 6 had monoclonal gammapathy [10][11][12][13]17] and 2 had macroglobulinemia [14,18]. In 9 of these 18 cases [8,10-14, 17, 18], insufficient data were available to evaluate and further subclassify the glomerular lesions.…”