A rare clinical case of combined kidney damage associated with IgG4-associated systemic disease

IgG4-associated disease is a systemic disease. Kidney damage is often noted in the form of interstitial nephritis, obstructive nephropathy, less often -glomerulopathy (including membranous nephropathy). IgG4-associated disease was isolated as an independent nosological form in 2003, when signs of systemic damage (involvement of the biliary tract, salivary glands, retroperitoneal space) were found in patients with type 1 autoimmune pancreatitis, and in 2012 the first international nomenclature of the disease was proposed. The clinical manifestations of IgG4-associated disease are nonspecific and diverse, which determines the difficulties of differential diagnosis, including infections and tumors, and increases the period from the onset of the disease to diagnosis to an average of 2 years. In recent years, there has been a significant increase in the number of studies devoted to this pathology, and in 2019, experts from the European Antirheumatic League (EULAR) and the American College of Rheumatology (ACR) proposed classification criteria for IgG4-associated disease. IgG4-associated disease is more likely to develop in middle age and old age. The prevalence of the disease is higher among men than among women, although the frequency of various clinical forms may vary depending on age and gender. Thus, autoimmune pancreatitis type 1, retroperitoneal fibrosis, and tubulointerstitial nephritis are more common in men, and sialoadenitis, dacryoadenitis, and thyroiditis are more common in women. In this article, we present a clinical case of IgG4-associated systemic disease with a combined lesion of the tubulo-interstitial and glomerular compartments of kidney tissue with nephrotic syndrome as the only initial manifestation. Membranous nephropathy was confirmed by nephrobiopsy. The combination of IgG4 and tubulo-interstitial nephritis with membranous nephropathy is an extremely rare pathology. The nephrotic syndrome was completely resolved after treatment with immunosuppressants. Nephrobiopsy was crucial in the diagnosis of this condition, which allowed the patient to be prescribed proper and timely treatment.

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