Immunoglobulin G4-related Lung Disease Research Articles

IGG4-RELATED DISEASE PRESENTING AS A PULMONARY INFLAMMATORY PSEUDOTUMOR

IGG4-RELATED DISEASE PRESENTING AS A PULMONARY INFLAMMATORY PSEUDOTUMOR

A QUEST TO FIND AN AETIOLOGY FOR A PULMONARY CONSOLIDATION BEYOND INFECTIVE AND MALIGNANT CAUSES

TYPE: Case Report TOPIC: Chest Infections INTRODUCTION: We present a rare inflammatory cause of radiological consolidation found in a 67-year-old lady with unresolved pneumonia. CASE PRESENTATION: A 67-year-old lady was referred to the chest clinic with a persisting right middle lobe opacity on subsequent chest radiography 6 week's after the initial presentation with pneumonia. She had completed a course of co-amoxiclav with clarithromycin. A contrast-enhanced CT Chest demonstrated consolidation in the medial aspect of right middle lobe. Spirometry was consistent with mild restrictive defect. Blood tests showed raised IgG and IgA levels. Serum electrophoresis revealed polyclonal increase in γ-globulins. Her bronchoalveolar lavage was unremarkable. This prompted to investigate her with CT-guided biopsy which showed fibro-inflammatory cells. Alas IgG4-related lung disease was confirmed. She showed clinico-radiological improvement with a course of steroid. Figure: CXR and CT chest showing RML consolidation DISCUSSION: IgG4-related lung disease can be associated with a rare pneumonic presentation. It is an idiopathic multi system disorder characterised by presence of IgG4-positive lymphoplasmacytic infiltrates and storiform fibrosis along with raised serum IgG4. It's presentation is organ specific and its pulmonary presentation ranges from consolidation to interstitial disease, and lymph node enlargement. Among the five radiologic subtypes, alveolar consolidative and solid nodular type remain most common. It is typically steroid responsive. CONCLUSIONS: Although very rare but this case depicts importance of considering inflammatory pneumonia such as IgG4 related lung disease as a differential in unresolved pneumonia. Reference 1: Kamisawa T. IgG4-related disease. Lancet 2015; 385: 1460. Refernece 2: Kang, J. Long-term clinical course and outcomes of immunoglobulin G4-related lung disease. https://doi.org/10.1186/s12931-020-01542-6 DISCLOSURE: Nothing to declare.

A case of immunoglobulin G4-related lung disease with normal serum immunoglobulin G4 level and hybrid radiological findings

A case of immunoglobulin G4-related lung disease with normal serum immunoglobulin G4 level and hybrid radiological findings

Long-term clinical course and outcomes of immunoglobulin G4-related lung disease

BackgroundImmunoglobulin G4-related lung disease (IgG4-RLD) is the pulmonary manifestation of a systemic fibroinflammatory disease characterized by lymphoplasmacytic infiltration with an abundance of IgG4-positive plasma cells. Long-term clinical course and outcomes of IgG4-RLD remain unclear. We aimed to identify clinical characteristics, treatment outcomes, and longitudinal pulmonary function changes in patients with IgG4-RLD according to the radiologic classification.MethodsChest computed tomography findings of 37 subjects were classified into five subtypes: solid nodular, bronchovascular, alveolar interstitial, round ground glass opacity, and alveolar consolidative. Radiologic treatment outcomes and longitudinal pulmonary function changes were compared among the different radiologic subtypes.ResultsThe mean age of the subjects was 55.6 years, and 78.4% were male. Among the five radiologic subtypes, alveolar consolidative and solid nodular type were most common, accounting for approximately 29.7% each of the total cases. Prednisone with or without azathioprine was administered to 31 patients (median treatment duration 14 months). In the treated patients, serial images showed complete response or partial response in 77.4%. However, relapse was documented in 25.0% of those who showed complete or partial response. In patients whose longitudinal lung function data were available (n = 20), the lung function was found to be stable during follow-up. Alveolar consolidative type showed the highest complete response rate, whereas alveolar interstitial type showed the lowest response rate, either complete or partial.ConclusionsMost patients showed a favorable outcome with regards to radiologic improvement and maintenance of pulmonary function; however, the response differed according to the radiologic subtype.

Features of IgG4-related lung disease on 18F-FDG PET/computed tomography imaging.

The aim of the study was to summarize the features of immunoglobulin G4-related lung disease (IgG4-RLD) on fluorine 18-fluorodeoxyglucose (F-FDG) PET/computed tomography (CT). In this retrospective case series, 12 consecutive patients (9 men and 3 women, mean age 55.4 ± 13.7 years) with IgG4-RLD were included. The clinicopathological information and features of F-FDG PET/CT imaging were analyzed. Six (50%) patients had pulmonary involvement alone and six (50%) patients had extrapulmonary involvement with intense F-FDG uptake. Pulmonary manifestations included mass (25%, 3/12), solid nodule (solitary 25%, 3/12; multiple 50%, 6/12), multiple ground-glass opacities (GGOs) (50%, 6/12), thickening of alveolar interstitium (50%, 6/12), and thickening of bronchovascular bundle (33.3%, 4/12). The maximum standardized uptake value (SUVmax) of the solid nodules and masses, multiple GGOs, bronchovascular bundle and the thickening of septa was 4.0 ± 2.5, 2.3 ± 1.8, 1.4 ± 0.6, and 0.9 ± 0.5, respectively. The SUVmax statistically significant linear association with the diameter of masses or solid nodules (P value = 0.03), but no significant inverse linear association (P value = 0.06) with the concentration of serum IgG4 concentration. The image patterns of IgG4-RLD on F-FDG PET/CT are varying. Multiple pulmonary manifestations or multiple organ involvement, especially in combination with elevated levels of serum IgG and IgG4, may help to make the diagnosis. A potential major application of PET-CT would be evaluation of response to treatment, and the impact of PET/CT on IgG4-RLD management is worth investigating further in the future.

A case of eosinophilic granulomatosis with polyangiitis complicated with a similar condition to IgG4 related lung disease

BackgroundAtypical manifestations, such as elevated serum immunoglobulin-G4 (IgG4) and extra-pulmonary IgG4 positive plasmacyte infiltration, have been described in patients with eosinophilic granulomatosis with polyangiitis (EGPA), such complicated situation might not be readily differentiated from IgG4-related disease.Case presentationHere, we report an interesting and rare case of EGPA in a 41 year-old male with negative anti-neutrophil cytoplasmic antibodies (ANCAs), which showed abundant pulmonary IgG4-positive plasma cells infiltration and markedly elevated serum IgG4 levels without extra-pulmonary lesions of IgG4-related disease. The clinical characteristics hesitated us whether the diagnosis as EGPA overlapping with IgG4-related lung disease should be concluded because of the absence of storiform fibrosis and obliterative phlebitis with lymphoplasmacytic infiltration. The patient’s systemic symptoms, pulmonary lesions, blood eosinophils count / percentage, and serum IgG4 levels were significantly improved with immunosuppressive therapy.ConclusionsWe consider that the overlapping pathogenesis exists in the disease course of EGPA and IgG4-related disease, which may lead to interaction during the course of the diseases. And the utilization of diagnostic criteria for IgG4-related lung disease with the careful differentiation is needed in such cases.

Immunoglobulin G4-related lung disease in an adolescent patient

Immunoglobulin G4-related disease is a recently recognized systemic immune-mediated condition characterized by lymphoplasmacytic infiltrate in various organs with IgG4 positive plasma cells. Isolated lung involvement is rare, difficult to diagnose and can mimic primary lung malignancy on imaging. We report the case of an isolated IgG4-related interstitial lung disease in a 16-year-old asymptomatic male adolescent with incidentally found bilateral nodular lesions by chest radiograph. The patient underwent surgical interventions for assumption of malignancy. Serum IgG4 levels was normal. Н istological examination revealed significant lymphoplasmacytic infiltrate with lymphoid follicle formation, peribronchial fibrosis, vascular obliteration. Prominent interstitial IgG4 positive plasma cell infiltrate was identified by immunohistochemistry. Isolated IgG4-related lung disease should be taken into account as a possible differential diagnosis of mass-forming lesions, even when no other organ manifestation is clinically apparent at the time of diagnosis.

Immunoglobulin G4-related lung disease

Lung involvement in IgG-related diseases is poorly investigated. The current approach to IgG-related diseases including IgG-related lung disease was discussed in the article. The authors described recent classification and diagnostic criteria of IgG4-related lung disease, including radiological, serological and morphological parameters, and therapeutic approaches. Two clinical cases of IgG4-related disease with involvement of the lungs (organizing pneumonia), lacrimal glands (dacryoadenitis), kidneys (tubulointerstitial nephritis), eyes (iridocyclitis), and salivary glands (sialadenitis) were described in the article. Immunosuppressive therapy was successful in both cases.

Reprint of: Pathologic manifestations of Immunoglobulin(Ig)G4-related lung disease.

Immunoglobulin(Ig)G4-related disease (IgG4-RD) is a fibroinflammatory condition that can affect virtually any organ and usually presents as tumefactive lesions involving multiple sites. Characteristic histopathology of IgG4-RD consists of dense lymphoplasmacytic infiltrate, fibrosis (often in storiform pattern), and obliterative phlebitis, accompanied by tissue infiltration of IgG4-positive plasma cells with or without elevation of serum IgG4 level. Despite a general similarity in the morphologic manifestations of IgG4-RD, site-specific unique morphologic features have been described in some organs including the lung. Compared with other sites, pulmonary involvement by IgG4-RD has been recognized more recently, and lung biopsy interpretation for this condition is often challenging, as both a relative paucity of pathognomonic features and a plethora of overlapping findings with other fibroinflammatory processes of the lung. This review is focused on the morphologic spectrum of IgG4-related lung disease documented in the current literature and on the pertinent issues in the differential diagnoses with other conditions encountered in the lung.

Sequential CT Findings in Two Cases of Immunoglobulin G4-Related Lung Disease: Focused on Disease Progression

Sequential CT Findings in Two Cases of Immunoglobulin G4-Related Lung Disease: Focused on Disease Progression

CLINICAL COURSE AND OUTCOME OF IMMUNOGLOBULIN G4 RELATED LUNG DISEASE

RespirologyVolume 22, Issue S3 p. 41-42 Clinical Respiratory Medicine 2 CLINICAL COURSE AND OUTCOME OF IMMUNOGLOBULIN G4 RELATED LUNG DISEASE First published: 23 November 2017 https://doi.org/10.1111/resp.13206_100Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat No abstract is available for this article. Volume22, IssueS3Supplement: 22nd Congress of the Asian Pacific Society of Respirology, International Convention Centre, Sydney, Australia, 23–26 November 2017November 2017Pages 41-42 RelatedInformation

Immunoglobulin G4-related Lung Disease: A Disease with Many Different Faces

Immunoglobulin (Ig) G4-related lung disease is a fibroinflammatory entity that presents in protean ways. Diagnostically, IgG4-related lung disease requires a high clinical index of suspicion complemented by elevated serum IgG4 levels and⁄or biopsy that shows the characteristic pathological features. The disease is almost always responsive to systemic corticosteroids. However, relapse is common following their discontinuation. The authors present three cases to highlight the diverse clinical features, and to illustrate the diagnostic and therapeutic approaches to this disease.

Immunoglobulin G4-related Lung Disease Accompanied by Organizing Pneumonia

The full picture of immunoglobulin G4-related lung disease (IgG4-RLD) has not yet been elucidated. A 69-year-old man was referred to us with a more than 2-week history of productive cough and fatigue. Chest CT showed an airspace consolidation along the bronchovascular bundles. The pathological findings that were obtained from an open-lung biopsy showed both organizing pneumonia and interstitial pneumonia. Based on the established, comprehensive diagnostic criteria for IgG4-related disease (RD) as of 2011, this patient was given a definitive diagnosis of IgG4-RD. A further accumulation and analysis of those cases that concomitantly present with both IgG4-RLD and organizing pneumonia, like our patient, may contribute to the elucidation of the pathology of IgG4-RLD and the establishment of the disease spectrum.

Pathologic manifestations of Immunoglobulin(Ig)G4-related lung disease

Immunoglobulin(Ig)G4-related disease (IgG4-RD) is a fibroinflammatory condition that can affect virtually any organ and usually presents as tumefactive lesions involving multiple sites. Characteristic histopathology of IgG4-RD consists of dense lymphoplasmacytic infiltrate, fibrosis (often in storiform pattern), and obliterative phlebitis, accompanied by tissue infiltration of IgG4-positive plasma cells with or without elevation of serum IgG4 level. Despite a general similarity in the morphologic manifestations of IgG4-RD, site-specific unique morphologic features have been described in some organs including the lung. Compared with other sites, pulmonary involvement by IgG4-RD has been recognized more recently, and lung biopsy interpretation for this condition is often challenging, as both a relative paucity of pathognomonic features and a plethora of overlapping findings with other fibroinflammatory processes of the lung. This review is focused on the morphologic spectrum of IgG4-related lung disease documented in the current literature and on the pertinent issues in the differential diagnoses with other conditions encountered in the lung.

Clinical Features of Airway Involvement in IgG4-Related Lung Disease: Analysis of 6 Cases

PURPOSE: Immunoglobulin G4 (IgG4) - related lung disease shows various lesions in respiratory system including bilateral hilar lymphadenopathy (BHL), bronchial wall thickening, interstitial pneumonia, nodule and consolidation in the lung, and pleural effusion. In the study, we studied clinical features of airway involvement of IgG4 related lung disease by analysis of 6 cases.

Immunoglobulin G4–related Lung Disease: CT Findings with Pathologic Correlations

To retrospectively analyze radiologic findings of immunoglobulin G4 (IgG4)-related lung disease as correlated with pathologic specimens. This study was approved by the institutional review board, and all patients had consented to the use of their medical records for the purpose of research. This study included 13 patients with IgG4-related lung disease (nine men and four women; age range, 43-76 years). Computed tomographic (CT) findings were retrospectively analyzed with regard to the characteristics, shape, and distribution of the radiologic findings and were correlated with surgically resected or biopsy lung specimens in seven patients. Statistical analysis was not used in this study. On the basis of the predominant radiologic abnormality, IgG4-related lung disease could be categorized into four major subtypes: solid nodular type having a solitary nodular lesion that included a mass (four patients); round-shaped ground-glass opacity (GGO) type characterized by multiple round-shaped GGOs (two patients); alveolar interstitial type showing honeycombing, bronchiectasis, and diffuse GGO (two patients); and bronchovascular type showing thickening of bronchovascular bundles and interlobular septa (five patients). Pathologically, solitary nodular lesions consisted of diffuse lymphoplasmacytic infiltration with fibrosis. Thickened bronchovascular bundles or interlobular septa and GGO on CT images pathologically corresponded to lymphoplasmacytic infiltration and fibrosis in peribronchiolar or interlobular interstitium and alveolar interstitium, respectively. The radiologic findings of honeycombing corresponded to disrupted alveolar structures and dilated peripleural air spaces. IgG4-related lung disease manifested as four major categories of CT features. Pathologically, these features corresponded to IgG4-related sclerosing inflammation along the intrapulmonary connective tissue.