A rare case: IgG4-related chronic inflammatory disease with kidney involvement

Abstract

IgG4-related disease is an inflammatory, multisystemic disease that affects the immune system. The disease progresses to fibrosis due to inflammation. Retroperitoneal fibrosis is a serious complication. Pancreas, biliary tract, glands, thyroid, lymph nodes, etc. may be involved. Prognosis is usually subacute, and seen in middle age and advanced men. It is characterized histopathologically by IgG4 positive plasma cells, lymphoplasmocytic cell infiltration, and storiform fibrosis. In our case, we evaluated a patient who referred to our clinic from an external center with the complaints of generalized pain, itching, tearing and redness in eyes, involvement of bilateral large joints, and impaired renal function. Diagnosis, treatment and management of the disease are important. Response to glucocorticoid therapy is good.

Keywords: IgG4 related disease; acute kidney injury; tubulointerstitial nephritis.

FIGURE 1

Contrast‐enhanced abdominal computed tomography. Inflammation in pararectal adipose tissue.

FIGURE 2

Contrast‐enhanced abdominal computed tomography. Loss of corticomedullary separation and hypodense patchy areas in both kidneys.

FIGURE 3

Examples of pararectal lymphatic tissue biopsy. Lymphoid tissue samples with increased plasma cells in hematoxylin–eosin staining.

FIGURE 4

Kidney biopsy samples. IgG4‐secreting plasma cells with (A) hematoxylin–eosin staining, (B) Masson trichrome staining, (C) IgG4 immunohistochemical staining.