Intrathoracic Involvements of Immunoglobulin G4-Related Sclerosing Disease

Abstract

To investigate clinical and radiological features of IgG4-related disease (IgG4-RD) patients with intrathoracic involvement.A prospective cohort study was performed and IgG4-RD patients were enrolled from January 2011 to March 2015 in Peking Union Medical College Hospital, in which the clinical and radiological characteristics of IgG4-RD patients with intrathoracic involvement were summarized.Out of total 248 cases with IgG4-RD, 87 cases had intrathoracic lesions, including 58 male cases and 29 female cases, with average age of 54.19 ± 13.80 years. Hilar and mediastinal lymphadenopathy were the most common manifestations of IgG4-related intrathoracic disease, accounting for 52.9% (46/87). Other imaging findings of pulmonary disease included: solid nodular (25.3%), round-shaped ground-glass opacities (9.2%), alveolar-interstitial type (20.7%), bronchovascular type (23.0%), pleural effusion (4.6%), and pleural nodules or thickening (16.1%). Only 27 patients presented with respiratory symptoms, including cough, breathless, chest pain, and asthma. Compared with patients without intrathoracic disease, IgG4-related intrathoracic disease had higher IgG4 and C-reactive protein level, and higher incidence of allergy, fever, and multi-organ involvement. Most of lung interstitial disease, mediastinal mass, and bronchial thickening were sensitive to corticosteroid and immunosuppressant therapy, while 36.3% (8/22) of solitary nodular lesions were unresponsive to treatment. Eight patients were on no treatment, with 5 cases remained stable, 2 patients improved spontaneously, and 1 patient was lost follow-up.Intrathoracic lesions are not rare in patients with IgG4-RD, involving bronchial thickening, nodules, ground glass opacity, pleural thickening/effusion, lymphadenopathy, etc. Efficacy of corticosteroid and immunosuppressant therapy were noted in most of patients with lung interstitial disease, mediastinal mass, and bronchial thickening.

Intrathoracic Manifestations of IgG4-Related Disease

“…3 Zen et al reported that eight of nine patients with the nodular type had resection with no recurrence of pulmonary lesions. One case of the nodular type improved with corticosteroid treatment.…”

“…16 Other intrathoracic manifestations include involvement of the pleura, pericardium, vasculature (the aorta, coronary arteries), and hilar and mediastinal lymph nodes. 3,5 Hilar and mediastinal lymphadenopathy, usually bilateral, is the most common intrathoracic finding and has been reported in 52.9-78.3% of patients. 3,5 IgG4-RD pleural disease presents alone or concurrently with parenchymal disease.…”

“…3,5 Hilar and mediastinal lymphadenopathy, usually bilateral, is the most common intrathoracic finding and has been reported in 52.9-78.3% of patients. 3,5 IgG4-RD pleural disease presents alone or concurrently with parenchymal disease. Previously reported manifestations of IgG4-related pleural disease include pleural nodules, 8 pleural effusion, 17 and extensive adhesive fibrinous pleural thickening (focal and diffuse).…”

“…2 In the previously mentioned study by Zen et al, pulmonary involvement was noted at 9.5% (11/114) of patients. 4 A higher prevalence has been reported in studies in which there was systemic review of all imaging studies, 3,5 including one study with the goal to identify intrathoracic findings of IgG4-RD.…”

“…In a cohort of 248 IgG4-RD patients with baseline and follow-up imaging, Fei et al reported that IgG4-RD intrathoracic findings were seen in 35% of the patients. 3 Zen et al found that 14% of patients (16/114) had IgG4-RD intrathoracic involvement in a cross sectional study of these patients. 4 Fujinaga et al systematically reviewed diagnostic images from 90 patients with autoimmune idiopathic pancreatitis and noted that 51.2% of the patients had pulmonary findings.…”