Mesangial IgA Nephropathy and Idiopathic Nephrotic Syndrome

Abstract

A 17-year-old male presented with nephrotic syndrome associated with microscopic hematuria. Renal biopsy showed only minor glomerular abnormalities (light microscopy). Immunohistology demonstrated strong mesangial deposition of IgA. Electronmicroscopy disclosed widespread effacement of foot processes in combination with isolated osmiophilic mesangial deposits. The patient responded to standard corticosteroid therapy with complete disappearance of proteinuria. Microscopic hematuria, however, persisted. Five months after steroid therapy was stopped, the nephrotic syndrome relapsed. It was again steroid-responsive with persisting microhematuria. From clinical and morphological data we conclude that the patient has concomitant idiopathic nephrotic syndrome (minimal change glomerulonephritis) and mesangial IgA glomerulonephritis. The simultaneous presence of these two diseases may give some hint as to their pathogenesis. In both, abnormalities in T cell regulation have been found. If these were indeed involved in the pathogenesis of the two glomerular diseases, a higher than expected probability for the two entities to coexist in the same patient is to be expected.

Mucosal Immunity in Primary Glomerulonephritis: II. Study of the Serum IgA Subclass Repertoire to Food and Airborne Antigens

“…We have recently shown that the increase in salivary IgAl and neopterin was not specific for IgA GN but was also observed in MGN and INS, suggesting an activation of the lymphoid system at the mucosal site in these nephrop athies, too [19]; an increase in total IgA in nasal washings of patients with glomerulopathies other than IgA GN was also reported by Yagame [25]. Furthermore, the find ing in INS of an increase in both IgAl and IgA2 at mucosal sites [19], and the detection in our study of high serum levels of specific IgA to environmental antigens strengthen the hypothesis that steroid-responsive ne phrotic syndrome with concomitant IgA deposits should not be considered as a distinct syndrome but rather a minimal-change disease with nonspecific mesangial trapping of IgA [26][27][28][29][30], A similar mechanism might be considered in MGN associated with IgA deposits [31].…”

IgA Nephropathy with Minimal Change Disease

“…The single pathologic finding that correlated with the presentation of full NS was extensive (median=90%) foot process effacement. The clinical and biopsy findings in these 17 patients, as well as similar cases that have been previously reported (4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17), are most consistent with mild IgAN with superimposed MCD.…”

“…Many of the patients are rapidly responsive to treatment with corticosteroids. Such cases are uncommon but have been presented as case reports and in small series over the last 30 years (4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17). Some of these reports have suggested that the clinical and pathologic findings in these unusual cases of IgAN (4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17), as well as the clinical outcomes (4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17), are more typical of mild IgAN with superimposed MCD.…”

IgA Nephropathy and Nephrotic Syndrome

“…We have recently read with great interest the paper by Rambausek et al [1], During the period from 1981 to 1985, IgA nephropathy was diagnosed in 63 patients who ac count for 14.6% of all biopsy-confirmed cases with pri mary glomerular disease in our hospital. Nephrotic syn drome was present in 8 patients (12.7%).…”