A clinical case of IgG4-releated disease
https://doi.org/10.28996/1680-4422-2018-1-92-99
Abstract
IgG4-associated disease (IgG4-AD) is a systemic disease characterized by inflammation and fibrosis that affects almost all systems and organs. Humoral and cellular immunity plays some role in the pathophysiology of IgG4-AD, but the mechanism of this process is currently not fully understood. The most frequent targets are the organs of the biliodigestive zone and kidneys. IgG4-associated tubulo-interstitial nephritis (TIN) is the most common variant of kidney damage associated with this disease. The article presents a case of IgG4-AD in a young patient which debuted with diabetes mellitus and acute renal injury. The diagnosis of IgG4-AD was verified morphologically: the characteristic dense interstitial infiltration represented by plasma cells with an increase in the number of IgG4-positive cells were detected in kidney biopsy. Taking into account the obtained data, the patient was diagnosed with the development of IgG4-associated renal lesions in the form of acute TIN and pancreatic gland in the form of autoimmune pancreatitis with the formation of secondary diabetes mellitus. According to the international standards of IgG4-AD therapy, systemic glucocorticoids were prescribed. As the result, there was a complete restoration of renal function, but a normalization of endocrine function of the pancreas was no achieved.
About the Authors
E. V. Voloshinova
Chair of hospital therapy, V.I. Razumovsky Saratov State Medical University
Russian Federation
Russian Federation
O. A. Vorobyeva
National Center of Clinical Morphological Diagnostics
Russian Federation
Russian Federation
K. N. Tsaturova
Chair of hospital therapy, V.I. Razumovsky Saratov State Medical University
Russian Federation
Russian Federation
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Review
For citations:
Voloshinova E.V., Vorobyeva O.A., Tsaturova K.N. A clinical case of IgG4-releated disease. Nephrology and Dialysis. 2018;20(1):92-99. (In Russ.) https://doi.org/10.28996/1680-4422-2018-1-92-99
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