Home
|
Inbox
|
Search
|
View Abstract
Subtotal Pleurectomy for IgG4 Related Pleural Disease Manifesting as Pleural Thickening
Description
.abstract img { width:300px !important; height:auto; display:block; text-align:center; margin-top:10px } .abstract { overflow-x:scroll } .abstract table { width:100%; display:block; border:hidden; border-collapse: collapse; margin-top:10px } .abstract td, th { border-top: 1px solid #ddd; padding: 4px 8px; } .abstract tbody tr:nth-child(even) td { background-color: #efefef; } .abstract a { overflow-wrap: break-word; word-wrap: break-word; }
A3145 - Subtotal Pleurectomy for IgG4 Related Pleural Disease Manifesting as Pleural Thickening
Author Block: S. Kuperberg1, P. Parayno2, N. R. Desai1; 1Interventional Pulmonology, Chicago Chest Center, Elk Grove Village, IL, United States, 2Department of Pathology, Amita Health, Alexian Brothers Hospital, Elk Grove Village, IL, United States.
Introduction: Thickening of the parietal pleura can develop secondary to malignancy, however inflammatory diseases of the pleural space are also common causes. IgG4-related disease has multiple pulmonary manifestations, including pleural effusion. Significant thickening of the pleura requiring definitive surgical intervention has rarely been reported.
Case Presentation: A 43-year-old male smoker presented with pleuritic chest pain and dyspnea for two months. Previous thoracenteses for pleural effusions were attributed to PE. Vital signs and lung exam were unremarkable. Thoracic CT revealed pleural thickening of the right hemi-thorax with a focus of gas. Serology was negative for ANA and ESR. Levofloxacin did not improve his symptoms. Thoracic Surgery subsequently performed Video Assisted Thoracoscopic Surgery (VATS), subtotal pleurectomy, pleural biopsy, decortication, and excision of a pleural peel. Pathology revealed marked fibrosis and chronic lymphoplasmacytic inflammation. Plasma cells were positive for features of autoimmune IgG4 related disease. Additional serology showed abnormal elevation of IgG subtypes, including the IgG4 subclass (271mg/dL, normal range 1-123 mg/dL). The patient’s symptoms improved dramatically after surgery.
Discussion: IgG4-related disease is an uncommon autoimmune, fibro-inflammatory disease affecting multiple organ systems. In the lung, it may present with nodules, ground glass opacities, or masses (1). Histopathology of IgG4 related pulmonary lesions reveals lymphoplasmacytic infiltration, fibrosis, and obliterative vasculopathy (1). The differential diagnosis includes lymphoproliferative malignancy, and benign conditions such as Castleman Disease, Lymphomatoid Granulomatosis and Granulomatosis with Polyangiitis. Pulmonary symptoms are typically responsive to corticosteroids. In our patient, surgical biopsy of the pleura correlated with serologic diagnosis, confirming IgG4-related disease. While pleural effusions occurring in isolation is a rare manifestation of this condition (2, 3), pleural thickening is even more uncommon. Our case describes a unique presentation of Ig-G4 related autoimmune disease involving unilateral pleural thickening improving with definitive surgical intervention. Although steroids are usually utilized, surgery provided significant relief for our patient. This case underscores that an index of suspicion should be held for autoimmune conditions causing thickening of the pleura, and that prompt surgical referral is often invaluable.
1. Campbell SN, Rubio E, Loschner AL. Clinical review of pulmonary manifestations of IgG4-related disease. Ann Am Thorac Soc. 2014 Nov;11(9):1466-75
2. Choi JH, Sim JK, Oh JY, et al. A Case of IgG4-Related Disease Presenting as Massive Pleural Effusion and Thrombophlebitis. Tuberc Respir Dis (Seoul). 2014 Apr;76(4):179-83.
3. Gajewska ME, Rychwicka-Kielek BA, Sørensen K, et al Immunoglobulin G4-related pleuritis - A case report. Respir Med Case Rep. 2016 May 24;19:18-20
Author Block: S. Kuperberg1, P. Parayno2, N. R. Desai1; 1Interventional Pulmonology, Chicago Chest Center, Elk Grove Village, IL, United States, 2Department of Pathology, Amita Health, Alexian Brothers Hospital, Elk Grove Village, IL, United States.
Introduction: Thickening of the parietal pleura can develop secondary to malignancy, however inflammatory diseases of the pleural space are also common causes. IgG4-related disease has multiple pulmonary manifestations, including pleural effusion. Significant thickening of the pleura requiring definitive surgical intervention has rarely been reported.
Case Presentation: A 43-year-old male smoker presented with pleuritic chest pain and dyspnea for two months. Previous thoracenteses for pleural effusions were attributed to PE. Vital signs and lung exam were unremarkable. Thoracic CT revealed pleural thickening of the right hemi-thorax with a focus of gas. Serology was negative for ANA and ESR. Levofloxacin did not improve his symptoms. Thoracic Surgery subsequently performed Video Assisted Thoracoscopic Surgery (VATS), subtotal pleurectomy, pleural biopsy, decortication, and excision of a pleural peel. Pathology revealed marked fibrosis and chronic lymphoplasmacytic inflammation. Plasma cells were positive for features of autoimmune IgG4 related disease. Additional serology showed abnormal elevation of IgG subtypes, including the IgG4 subclass (271mg/dL, normal range 1-123 mg/dL). The patient’s symptoms improved dramatically after surgery.
Discussion: IgG4-related disease is an uncommon autoimmune, fibro-inflammatory disease affecting multiple organ systems. In the lung, it may present with nodules, ground glass opacities, or masses (1). Histopathology of IgG4 related pulmonary lesions reveals lymphoplasmacytic infiltration, fibrosis, and obliterative vasculopathy (1). The differential diagnosis includes lymphoproliferative malignancy, and benign conditions such as Castleman Disease, Lymphomatoid Granulomatosis and Granulomatosis with Polyangiitis. Pulmonary symptoms are typically responsive to corticosteroids. In our patient, surgical biopsy of the pleura correlated with serologic diagnosis, confirming IgG4-related disease. While pleural effusions occurring in isolation is a rare manifestation of this condition (2, 3), pleural thickening is even more uncommon. Our case describes a unique presentation of Ig-G4 related autoimmune disease involving unilateral pleural thickening improving with definitive surgical intervention. Although steroids are usually utilized, surgery provided significant relief for our patient. This case underscores that an index of suspicion should be held for autoimmune conditions causing thickening of the pleura, and that prompt surgical referral is often invaluable.
1. Campbell SN, Rubio E, Loschner AL. Clinical review of pulmonary manifestations of IgG4-related disease. Ann Am Thorac Soc. 2014 Nov;11(9):1466-75
2. Choi JH, Sim JK, Oh JY, et al. A Case of IgG4-Related Disease Presenting as Massive Pleural Effusion and Thrombophlebitis. Tuberc Respir Dis (Seoul). 2014 Apr;76(4):179-83.
3. Gajewska ME, Rychwicka-Kielek BA, Sørensen K, et al Immunoglobulin G4-related pleuritis - A case report. Respir Med Case Rep. 2016 May 24;19:18-20
|
Home
|
Inbox
|
Search
|