Akademik Veri Yönetim Sistemi
ULUSLARARASI KATILIMLI 40. RADYOLOJİ KONGRESİ - 2019 TÜRKRAD 2019, Antalya, Türkiye, 5 - 10 Kasım 2019, ss.846
Introduction: Immunoglobulin G4-related disease (IgG4RD) is arecently defined entity and its pathogenesis has not been elucidated yet. In the literature, lung involvement associated with IgG4RD has been described in a small number of cases. Herein, we present chest computed tomography (CT) findings of a case diagnosed with IgG4related lung disease.
Case Report: A 36-year-old male patient admitted to the hospital with (acute?/chronic?) abdominal pain. Contrast-enhanced abdomen CT revealed a mass like lesion on the pancreas head and Whipple operation was performed (Figure 1). Histopathological results were reported as chronic pancreatitis. During the follow-up period of the patient; hypothyroidism and asthma-like symptoms developed. Cervical lymphadenopathies and increased volume of salivary glands were detected with ultrasonography. Chronic inflammation was found in the salivary gland biopsy. Paranasal sinus CT revealed pan sinusitis. The blood IgG4 value was determined five times above normal values. The patient was diagnosed as IgG4RD. At this stage, contrast-free chest CT; scattered settled ground-glass areas, patchy consolidations, and nodular densities were found in both lungs (Figure 1). The areas of involvement in both lungs were predominantly peripheral. In addition, there are multiple lymph nodes in the mediastinum and in both axillae (Figure 2). With the treatment given for IgG4, it was observed that these chest CT findings were lost in both lungs. In addition, similar findings were observed again during the attack episodes of the disease after three months. The patient was diagnosed as IgG4RD related to lung involvement.
Discussion: Immunoglobulin G4-related disease (IgG4RD) is an immune-mediated condition comprised of a collection of disorders that share particular pathologic, serologic, radiologic and clinical features (1). The three major pathology features of IgG4RD are lymphoplasmacytic infiltration, storiform fibrosis and obliterative phlebitis (2). In addition, elevated serum concentrations of IgG4 are found in 60 to 70 percent of patients. The disease may cause involvement in many organs including pancreas, liver, biliary duct, kidneys, thyroid and salivary gland, central nervous system, lungs, retroperitoneum and lymph nodes (3). Lung involvement of IgG4RD is a very rare condition. Due to pulmonary involvement of the disease; solid nodules, round-shaped opacities, honeycombing, bronchiectasis, thickening of bronchovascular bundles and interlobular septae can be seen in chest CT. In our case; scattered settled ground-glass areas, patchy consolidations and nodular densities were found in both lungs. Although transbronchial biopsy was the definite diagnosis of lung involvement in IgG4RD; in our patient, the diagnosis was confirmed by the typical involvement of other organs and biopsy of the pancreas head and salivary glands in accordance with IgG4RD and the disappearance of lung findings with the response to treatment. In conclusion, IgG4RD is a rare disease with multisystemic involvement and lung involvement is infrequent. In the presence of findings in other systems, IgG4RD may be considered in the differential diagnosis of chest CT findings.
Anahtar kelimeler: Immunoglobulin G4-related disease, thorax, Computed Tomography