Diffuse Lung Disease |

IgG4-Related Lung Disease: A Case Report FREE TO VIEW

Yong Liu*, MD; Yongchang Sun, MD; Xiaofang Liu, MD; Ruie Feng, MD
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Beijing Tongren Hospital, Capital Medical University, Beijing, China

Chest. 2012;142(4_MeetingAbstracts):459A. doi:10.1378/chest.1389734
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SESSION TYPE: ILD Global Case Report Posters

PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM

INTRODUCTION: IgG4-ralated lung disease is a sclerosing disease combined with or without extrapulmonary involvement. Herein, we report one case of IgG4-RLD without other organ involvement and its radiographic features just mimicked diffused parenchymal lung disease.

CASE PRESENTATION: A 41-year-old male ,whose was asymptomatic, was admitted to our department because of diffuse opacities on regular X-ray examination. A CT scan was performed and revealed diffused ground-glass opacities(GGO) and reticular opacities distributed through both sides of lungs. His medical history and family history was unremarkable. He is a smoker with one half package per day for 10 years but cessation for 6 years. He had no history of environmental exposure to chemicals or dust. Physical examination was normal. The routine tests is in the normal range. ESR is 9mm/hr and CRP is 4ug/ml. Arterial blood analysis on room air revealed: PH 7.41, PaO2 78 mmHg, PaCO2 40 mmHg. The serum concentration of CEA was in the normal limits, as well as CA125, CA199, PSA and NSE. Pulmonary function tests were as follows: TLC, 99.2% predicted; VT, 154.1% predicted; FEV1.0, 83.8% predicted; FEV1.0/FVC ratio, 40.7%;DLco 67.8%,DLco/VA 100.3%. Diffused parenchymal lung disease is suspected and some autoimmune examination were performed. RF was 129 IU/ml(0-20), ANA was 1:100(<1:80) , others were all negative, including dsDNA, ANCA and ENA spectrum. Immunoglobulin analysis revealed: IgG 13.3g/L(7-17), IgA 2.94g/L(0.7-3.8), IgM 0.71g/L(0.6-2.5). So we think there is less possible of CTD-ILD. A bronchofibroscopy examination was performed and BALF analysis was as follows: total cell count, 462×106/L; L 23.69%, N 1.5%, E 0.5%, M 59.5%; CD4/CD8=1.0,cytopathologic examination is negative. The histopathological diagnosis is not identified because of the unsatisfied TBLB sample. VATS lung biopsy was performed in order to identify the nature: Massive lymphoplasma cell infiltration and collagen deposition along the alveolar septa and bronchovascular bundles were identified on HE staining; Obliterative phlebitis and thickeness of alveolar septa were observed. IgG4 immunostaining revealed predominant IgG4+ plasma cells. IgG4-related lung disease was diagnosed combined with elevation of serum IgG4 concentration(307mg/dl). Other examinations, such as serum amylase, lipase,abdominal CT scan and MRCP, were performed and there was no evidence of extrapulmonary involvement. The patient received oral prednisone at the dose of 30mg per day for one month and then tapered. Four months later a CT scan revealed that the GGOs disappeared and only some reticular opacities and honeycombing changes remained. The serum IgG4 concentration decreased to 199mg/dl.

DISCUSSION: IgG4-related lung disease is a rare disease, especially lung involvement alone. Twenty-one articles with 65cases of IgG4-related lung disease were acquired through PubMed search engine and 27 cases without extrapulmonary involvement were identified. Four radiographic patterns were identified by Inoue:solid nodules type,alveolar interstitial type,bronchovascular type and round-shaped GGO type. According to the classification above, the case we reported belongs to the alveolar interstitial type. The key of diagnosis is the histopathological features, especially the IgG4+/IgG+ plasma cell ratio and IgG4+ plasma cell count per HPF. The elevation of serum IgG4 also contributes to the diagnosis. Glucocorticosteroid is the favorable choice and a good response may be achieved. According to the literatures, the prognosis is good except one case relapsed one year after the cessation of prednisone.

CONCLUSIONS: Some kind of IgG4-RLD may mimicked as interstitial lung disease. Glucocorticosteroid is the favorable choice and a good response may be achieved.

1) Inoue D, Zen Y, Abo H, et al. Immunoglobulin G4-related lung disease: CT findings with pathologic correlations. Radiology 2009; 251:260-270

2) Umehara H, Okazaki K, Masaki Y, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol 2012

DISCLOSURE: The following authors have nothing to disclose: Yong Liu, Yongchang Sun, Xiaofang Liu, Ruie Feng

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Beijing Tongren Hospital, Capital Medical University, Beijing, China




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