Disparities between IgG4-related kidney disease and extrarenal IgG4-related disease in a case–control study based on 450 patients

Abstract

We aimed to compare the demographic, clinical and laboratory characteristics between IgG4-related kidney disease (IgG4-RKD+) and extrarenal IgG4-related disease (IgG4-RKD−) in a large Chinese cohort, as well as describing the radiological and pathological features of IgG4-RKD+. We retrospectively analyzed the medical records of 470 IgG4-related disease (IgG4-RD) patients at Peking University People’s Hospital from January 2004 to January 2020. The demographic, clinical, laboratory, radiological and pathological characteristics between IgG4-RKD+ and IgG4-RKD− were compared. Twenty IgG4-RD patients who had definite etiology of renal impairment including diabetes, hypertension and etc. were excluded. Among the remained 450 IgG4-RD patients, 53 were diagnosed with IgG4-RKD+ . IgG4-RKD+ patients had older age at onset and at diagnosis. Male to female ratio of IgG4-RKD+ patients is significantly higher. In the IgG4-RKD+ group, the most commonly involved organs were salivary gland, lymph nodes and pancreas. It was found that renal function was impaired in approximately 40% of IgG4-RKD+ patients. The most common imaging finding is multiple, often bilateral, hypodense lesions. Male sex, more than three organs involved, and low serum C3 level were risk factors for IgG4-RKD+ in IgG4-RD patients. These findings indicate potential differences in pathogenesis of these two phenotypes.

Gastroenterological Manifestations of Immunoglobulin G Subclass 4-Related Disease—Epidemiology, Clinical Manifestations, Diagnosis and Treatment

“…Also known as immunoglobulin G4-related hepatobiliary disease, this condition manifests as glucocorticoid-responsive cholangitis of the extrahepatic and perihilar bile ducts, often associated with type 1 AIP. The symptomatology presents as jaundice, pruritus, weight loss and abdominal pain [ 16 ]. However, inflammatory pseudotumors of the liver and biliary cirrhosis can also develop as some late manifestations of this condition [ 9 , 15 ].…”

“…In cases of recurrence or relapse, azathioprine, methotrexate or rituximab can be used. In severe cases of SSC with significant obstruction of the bile ducts, surgical intervention or endoscopic procedures to clear bile flow may be performed [ 9 , 16 , 25 ].…”

Clinical features and relapse risks factors of immunoglobulin G4–related disease: a single-center retrospective study

“…Studies have shown that female patients are more likely to have super cial organs like the lacrimal glands and salivary glands involved, while visceral organs such as the pancreas and retroperitoneum are more predominantly involved in male patients [19] . There are also some studies reporting that common organs (e.g., salivary glands) are more often involved in women [20] , while rarer organ involvement (e.g., broseptitis, lung, and kidneys) mainly occurs in men [21,22] . In a 10-year study, the rate of IgG4-related ocular disease was similar between the sexes [23] .…”

“…In addition, the involvement of different organs is linked, as shown by simultaneous or sequential involvement of the lacrimal glands, salivary glands, and lymph nodes. Kidney involvement is rare but closely related to the involvement of more commonly infected organs like the salivary glands, lymph nodes, and pancreas [22] . Renal biopsy may be a good diagnostic tool for diagnosing renal involvement in patients with IgG4-related lymphadenopathy, IgG4-related sialadenitis, or autoimmune pancreatitis.…”

Renal and Urinary Tract Involvement in Fibrosclerosing or Fibroinflammatory Diseases: A Narrative Review

“…In the presence of a diagnosis of IgG4-RD, male sex, older age, involvement of three or more organs, a higher index of disease activity, and low serum C3 level increase the probability of developing or having already developed renal involvement [38].…”