Three cases of immunoglobulin G4-related respiratory disease with uncommon imaging findings

Immunoglobulin G4-related disease (IgG4-RD) is a rare multisystemic idiopathic fibroinflammatory disorder. The rare form of IgG4-RD with isolated thorax involvement is called immunoglobulin G4-related respiratory disease (IgG4-RRD). IgG4-RRD, which is reported in a limited number of cases in the literature, can be categorized into four types on the prevalent chest computed tomography (CCT) findings: solid nodular, round-shaped ground-glass opacity, alveolar interstitial, and bronchovascular. Solid nodular form of IgG4-RRD with mass-like lesions is sporadic and described in the literature with a small number of case reports.

We aim to present the radiologic, pathologic, and clinical findings of three cases of IgG4-RRD mimicking lung cancer.

In all three patients, IgG4-RRD occurred with mass-like lesions in the thorax. In case-1 and 2, CCT showed multiple, nodular lesions and multiple mediastinal lymph nodes. On positron emission tomography with 2-deoxy-2-[fluorine-18] fluoro- D-glucose integrated with computed tomography (18F-FDG PET/CT), the masses showed increased 18F-FDG uptake in case-2 and 3. The gold standard histopathological verification for IgG4-RRD was provided for all cases.

IgG4-RD is an immune-mediated condition comprised of a collection of disorders that share particular pathologic, radiologic, serologic, and clinical features. Isolated IgG4-RRD is rarely seen and is available in the literature as case reports. IgG4-RRD, which can make lung involvement in different patterns, rarely appears with mass-like lesions. Still, IgG4-RRD must be considered in the differential diagnosis of mass lesions detected in CCT. Laboratory, radiological, and histopathological findings of the disease should be evaluated together for an accurate diagnosis.