ADOPTING ORPHAN DISEASES RARE INTERSTITIAL LUNG DISEASES

adoptingorphandiseaseskapak

THORACIC INVOLVEMENT IN IGG4-RELATED DISEASES

Merve Sinem Oğuz1 Züleyha Bingöl2

1İskenderun State Hospital, Department of Chest Diseases, Hatay, Türkiye
2İstanbul University, Faculty of Medicine, Department of Chest Diseases, İstanbul, Türkiye

Oguz MS, Bingol Z. Thoracic Involvement in IgG4-Related Diseases. In: Altinisik G, McCormack FX, editors. Adopting Orphan Diseases: Rare Interstitial Lung Diseases. 1st ed. Ankara: Türkiye Klinikleri; 2025. p.171-185.

ABSTRACT

Immunoglobulin G4-related disease (IgG4-RD) is a rare, systemic, immune-mediated, fibroinflammatory disorder that can affect multiple organs. In recent years, thoracic involvement has been increasingly recognized. The disease typically occurs in middle-aged and older men; in patients with thoracic involvement, the diagnosis is most often established around 60 years of age. Concomitant extrathoracic organ disease raises diagnostic suspicion. Thoracic involvement may encompass the lungs, mediastinal and hilar lymph nodes, pleura, and airways, as well as vascular structures and the posterior mediastinum. The most frequent respiratory manifestations are nonspecific, including dyspnea, cough, and chest pain, although some patients remain asymptomatic.

Diagnosis requires integration of clinical findings with imaging, serology, and histopathology. Imaging features in thoracic IgG4-RD are non-pathognomonic and heterogeneous. Common radiologic findings include mediastinal lymphadenopathy, pulmonary nodules, bronchovascular bundle thickening, and pleural abnormalities. Serologically, elevated serum IgG4 concentrations (typically ≥135 mg/dL) and increased circulating plasmablast counts (>900/mL) are characteristic; however, normal serum IgG4 does not exclude the diagnosis. The distinctive histopathologic and immunohistochemical hallmarks include a dense lymphoplasmacytic infiltrate, storiform-pattern fibrosis, obliterative phlebitis or arteritis, and an increased IgG4+/IgG+ plasma-cell ratio (>40%), together with high tissue counts of IgG4+ plasma cells. Although thresholds vary by organ, for pulmonary and pleural disease the recommended cutoffs are >50 IgG4+ cells per high-power field (HPF) in surgical biopsies and >20 cells/HPF in nonsurgical biopsies.

Treatment is indicated for symptomatic thoracic disease; asymptomatic individuals (such as those with isolated mediastinal lymphadenopathy or incidentally detected small lung nodules) may be managed with close surveillance. Corticosteroids remain first-line therapy, whereas rituximab or inebilizumab are effective options for refractory or steroid-dependent cases. Although initial therapeutic responses are often robust, relapses are common, underscoring the need for structured, long-term follow-up that includes clinical and laboratory assessment and interval imaging when appropriate. In the diagnostic workup of IgG4-RD, exclusion of alternative diagnoses is fundamental. Accordingly, multidisciplinary evaluation improves diagnostic accuracy, excludes mimickers, and guides early treatment planning. Future research should focus on refining diagnostic criteria, identifying prognostic biomarkers, and developing targeted, durable therapies for IgG4-RD with thoracic involvement.

Keywords: Immunoglobulin G4-related disease; Rare interstitial lung diseases; Thoracic diseases

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