Background/Purpose: The intrathoracic manifestations of IgG4-related disease (IgG4-RD) have been described in only a limited number of patients. No prior studies have provided detailed descriptions of the clinical, radiological, and pathology features of IgG4-related lung disease (IgG4-RLD) in patients from North America.

Methods: We reviewed a cohort of IgG4-RD patients to identify those with intrathoracic disease manifestations. The medical records of 143 subjects with clinicopathologic diagnoses of IgG4-RD were screened to determine which had undergone computed tomography (CT), positron emission tomography (PET), or magnetic resonance (MR) imaging of the thorax. All thoracic imaging studies were reviewed by two thoracic radiologists to document intrathoracic manifestations of IgG4-RD. Data pertaining to patient clinical characteristics, radiologic features, and pathology were collected and analyzed.

Results: Sixty-three subjects had undergone CT, PET, or MRI studies of the chest. Of these, 36 had intrathoracic manifestations consistent with IgG4-RD. Twenty-seven (75%) of these subjects had involvement of the lung, pleura, or both. Intrathoracic manifestations typically occurred in the setting of systemic IgG4-RD, but 75% of the patients with intrathoracic disease had no respiratory symptoms at the time of imaging. Mediastinal/hilar lymphadenopathy, detected in 25 patients (69%), was the most common intrathoracic imaging manifestation. Airway disease consisting of bronchial wall thickening was also commonly noted (50%, 18 patients). Pulmonary nodules were identified in 12 patients (33%). Interlobular septal thickening (28%, 10 patients) and subpleural reticulation (11%, 4 patients) were also observed. Airspace disease consisting of consolidation was also noted in six patients (17%) and ground glass opacity in ten patients (28%). Other intrathoracic manifestations included pleural disease (25%, 9 patients), pericardial disease (8%, 3 patients), vascular involvement (aortitis, pulmonary arteritis, coronary arteritis) (14%, 5 patients), and paravertebral masses (22%, 8 patients). Follow-up with regard to treatment response of intrathoracic findings (defined as improved/stable clinical or radiological findings) to prednisone was available in 12 patients. On prednisone, 8 of 12 patients (66.7%) demonstrated overall improvement or stability of their disease. Follow-up to rituximab treatment was available in 11 cases. On rituximab, ten out of 11 patients (90.1%) demonstrated improvement or stable disease.

Conclusion: In conclusion, the heterogeneity of intrathoracic IgG4-RD manifestations pose significant challenges in diagnosis. The radiologic findings in the lung, in particular, are more diverse than any other organ commonly affected by this disease. Many patients with IgG4-RLD are relatively asymptomatic despite substantial burdens of disease within the lung. The intrathoracic manifestations of IgG4-RD responded well to glucocorticoids and B cell depletion. Because effective treatment is available for IgG4-RD, detection and treatment of disease this asymptomatic stage may lead to better patient outcomes.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/intrathoracic-manifestations-of-igg4-related-disease-findings-in-a-cohort-study-from-north-america/