Abstract:
IgG4 related disease appears as a component of systemic, immune mediated fibro-inflammatory condition implicating diverse organs. Incrimination of renal parenchyma manifests as an acute or chronic interstitial nephritis delineating a lymphoid and plasma cell rich inflammatory infiltrate with predominance of IgG4+ plasma cells. Besides, tubules and interstitium display deposition of immune complexes along with frequent occurrence of fibrosis configuring tumorous masses or a storiform pattern. Incrimination of glomeruli is variable and essentially emerges as membranous nephropathy. Pathognomonic features as chronic interstitial nephritis with an intense lymphoplasmacytic inflammatory infiltrate with preponderant IgG4+ plasma cells and foci of storiform fibrosis are discerned. Generally, storiform fibrosis is associated with diverse systemic manifestations of IgG4 related disease. The condition may recapitulate diverse causes of membranous nephropathy and allied patterns of cellular injury. Enhanced interstitial subtype of collagen bundles may be observed. Few lesions may be classified contingent to fibrosis and configured dissemination of collagen bundles.