IgG4-related pulmonary disease

BACKGROUND: IgG4-related disease is a systemic fibroinflammatory disease that is mainly seen in older men, and involves multiple organs, such as the pancreas and lungs. However, 75% of patients with IgG4-related lung disease are asymptomatic (if they are symptomatic, they mainly complain of nasal congestion, rhinorrhoea, chest pain, and cough) and are incidentally diagnosed through chest computed tomograph. Although, nodules in the airway and bronchial wall thickening are criteria for diagnosis, it is important that nodules have been reported in peripheral airways in several cases and rarely in the central airway...

BACKGROUND: IgG4-related disease (IgG4RD) is a rare fibroinflammatory disease with multiorgan involvement. It presents insidiously over several years and can be a diagnostic enigma. Delays in diagnosis occur due to failure to consider IgG4 as a differential diagnosis, atypical presentations, and an insidious clinical course. CASE PRESENTATION: We report the case of a 70-year-old Sri Lankan man with pulmonary, renal, and neurological involvement of IgG4-related disease...

Immunoglobulin G4-related disease (IgG4-RD) is a systemic condition known to affect multiple organ systems. While its manifestations are diverse, pulmonary involvement, especially of the pleura, remains less common. We report the case of a 99-year-old Saudi male with a medical history of diabetes mellitus, chronic kidney disease, and hypertension. He presented with dyspnea and syncope, with radiological findings revealing pleural effusion and a mass in the right hemidiaphragm. Laboratory investigations highlighted elevated serum IgG4 levels, and histopathological evaluation confirmed the diagnosis of IgG4-RD...

BACKGROUND: IgG4-related disease (IgG4-RD) is an inflammation-mediated autoimmune disease characterized by infiltration of IgG4 plasma cells in target organs, storiform fibrosis and obliterative phlebitis, accompanied by or without elevated serum IgG4 concentrations. Multiple sites can be involved, including large vessels. Coronary and pulmonary arteries are less involved, while simultaneous involvement of coronary and pulmonary arteries is less reported. This case is unique in terms of simultaneous involvement of coronary and pulmonary arteries in a female patient with possible IgG4-RD and the first review of relevant domestic literature...

IgG4-related lung disease is a diagnostic challenge as the presenting patterns can mimic other commonly seen pulmonary diseases like infections, interstitial pneumonia, malignancy, etc. The diagnosis of IgG4-related disease requires a correlation of clinical, radiological, biochemical, and histopathological features. Especially, an adequate tissue sample is necessary to diagnose this disease confidently. Conventionally surgical lung biopsy was needed for histopathology. But with the availability of the new less invasive technique-transbronchial cryo lung biopsy, adequate tissue can be obtained to clinch the diagnosis via bronchoscopy, thereby avoiding surgery...

Immunoglobulin G4 (IgG4)-related lung disease can have various clinical courses. To our knowledge, reports of IgG4-related lung disease with waxing and waning pulmonary infiltrates only are very rare. A few lung nodules and ground glass opacities were incidentally found in a pre-operative evaluation in a 36-year-old female. The lung lesions showed waxing and waning in the follow-up chest CT. She underwent a surgical biopsy, and IgG4-related lung disease was confirmed.

OBJECTIVES: DnaJ homolog subfamily B member 9 (DNAJB9) is a co-chaperone protein that governs the functions and integrity of cells. In immunoglobulin G4-related disease (IgG4-RD), DNAJB9 was shown to be upregulated in plasma cells, but its immunohistochemical expression has never been explored. This pilot study aims to investigate the immunohistochemical distribution and intensity of DNAJB9 in IgG4-RD tissue specimens. METHODS: Patients with definite IgG4-RD and normal tissue controls were selected for anti-DNAJB9 immunohistochemistry, applying a semi-quantitative staining intensity score...

The recognition of immunoglobulin G4-related disease (IgG4-RD) as an entity in the pancreaticobiliary tract was followed by a slew of papers describing inflammation and fibrosis containing IgG4-positive plasma cells in a variety of sites including the respiratory tract, leading to the hypothesis that these abnormalities were attributable to IgG4-RD. Predictably, pathologists began to see requests from clinicians to perform IgG4 immunohistochemistry in lung biopsies "to rule out IgG4-RD". Several years later, the notion that IgG4-RD would prove to be the underlying cause of a wide array of fibroinflammatory lesions in the lung has not panned out as promised...

BACKGROUND: Idiopathic multicentric Castleman disease (iMCD) is a rare polyclonal lymphoproliferative disease often associated with pulmonary involvement. Recently, transbronchial lung cryobiopsy (TBLC) has been reported to be useful for the diagnosis of diffuse interstitial lung disease. However, there have been no reports of pathological assessment of TBLC for iMCD. METHOD: To clarify the efficacy of TBLC in the diagnosis of iMCD, we retrospectively reviewed four iMCD patients who had undergone both TBLC and surgical lung biopsy (SLB)...

INTRODUCTION: Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated condition associated with fibroinflammatory lesions that can occur at almost any anatomical site. It often presents as a multiorgan disease that may mimic malignancy, infection, or other immune-mediated conditions. Autoimmune pancreatitis (AIP) type 1 is the most prominent manifestation of IgG4-RD in the digestive tract, with common extra-pancreatic inflammation. We present the first patient with AIP and involvement of the testicles and nasal cavity...

IgG4-related disease (IgG4-RD) is a systemic autoimmune disease characterized by the infiltration of a large number of IgG4+ plasma cells, neoplastic lesions in the affected tissues, and a sharp increase in the concentration of serum IgG4. IgG4-RD is a rare and novel disease involving multiple organs with various clinical manifestations. Understanding and studying the pulmonary manifestations of IgG4-RD is critical for improving diagnosis, treatment, and prognosis. However, lung involvement alone is less common...

Lung cancer is the most diagnosed cancer worldwide. Many non-malignant pulmonary lesions, such as tuberculosis, fungal infection, organizing pneumonia, inflammatory myofibroblastic tumor, and IgG4 disease, can mimic lung cancer due to their overlapping morphological appearance on imaging. These benign entities with minor differentiating imaging clues may go unnoticed in a high-volume cancer institution, leading to over-investigation that may result in repeated biopsies, pointless wedge resections, and related morbidities...

We present a patient with IgG4-related disease (IgG4-RD) that developed after receiving extra-periosteal paraffin-embedded therapy for the treatment of pulmonary tuberculosis. The patient showed clinicopathological features consistent with IgG4-RD, including the enlargement of affected organs (salivary glands, lymph nodes, and retroperitoneal soft tissue mass), elevation of serum IgG4 levels, and infiltration of IgG4-positive plasma cells. The presence of reactive granulomas with foreign body giant cells (FBGCs) surrounding the paraffin-filled site suggested a type 2 helper T (Th2)-dominant immune response induced by the implanted biomaterial...

A 65-year-old man presented with apparent bronchopneumonia. After treatment with antibiotics, he showed eosinophilia. Computed tomography (CT) imaging revealed bilateral consolidation, ground-glass opacities with nodular consolidations and pleural effusion. Lung biopsy showed organizing pneumonia with lymphoplasmacytic infiltration in alveolar septa and in thickened pleura and interlobular septa. All pulmonary abnormalities spontaneously went into remission within 12-months. At 73 years old, a follow-up CT scan revealed small nodules in both lungs and the review of head CT scan showed thickening of pituitary stalk in studying prolonged headache...

INTRODUCTION: IgG4-related disease is a poorly understood immune disorder. Its features include tumour-like swelling of involved organs, lymphoplasmacytic infiltrate with IgG4 positive plasma cells. IgG4-related lung disease can manifest radiologically as various types of pulmonary abnormalities, including mass-like lesions and pleural effusion, and it may mimic malignant disease. PRESENTATION OF CASE: A 76-year-old man was found to have a 4-mm ground glass opacity in the left lower lobe of the lung on follow-up chest CT after surgery for colon carcinoma...

BACKGROUND: Allergic bronchopulmonary aspergillosis (ABPA) is an immune-related pulmonary disease caused by sensitization of airway by Aspergillus fumigatus . The disease manifests as bronchial asthma and recurring pulmonary shadows, which may be associated with bronchiectasis. The diagnosis of ABPA mainly depends on serological, immunological, and imaging findings. Pathological examination is not necessary but may be required in atypical cases to exclude pulmonary tuberculosis, tumor, and other diseases through lung biopsy...

Immunoglobulin G4-related disease (IgG4-RD) is an immunomediated disease that can virtually affect any organ. Despite the pancreas being known as the most frequently involved organ, pulmonary and pleural IgG4-RD is being increasingly reported. The authors present two cases of IgG4-RD diagnosed in the same year, with different presentations and outcomes, in which the lung and pleural involvement were essential for the diagnosis. Recognizing IgG4-RD as a possible cause of chronic pleural effusion and/or thickening and lung abnormalities is important for an early diagnosis and prognosis improvement...

Tissue injury in nonmalignant human disease can develop from either disproportionate inflammation or exaggerated fibrotic responses. The molecular and cellular fundamental of these 2 processes, their impact on disease prognosis and the treatment concept deviates fundamentally. Consequently, the synchronous assessment and quantification of these 2 processes in vivo is extremely desirable. Although noninvasive molecular techniques such as 18 F-fluorodeoxyglucose PET offer insights into the degree of inflammatory activity, the assessment of the molecular dynamics of fibrosis remains challenging...

OBJECTIVES: Immunoglobulin G4-related diseases (IgG4-RD) is a rare autoimmune disease, and there is no specific diagnostic test for patients with lung involvement yet. This study aims to summarize the clinical characteristics of IgG4-RD with lung involvement and improve the understanding and diagnosis of this disease. METHODS: All patients diagnosed with IgG4-RD in the Second Xiangya Hospital from December 2014 to February 2022 were re-diagnosed according to the recommendations of Chinese Expert Consensus on the Diagnosis and Treatment of IGG4-Related Diseases in 2021...

BACKGROUND: IgG4-related disease (IgG4-RD) is a progressive and sometimes fatal disease that rarely affects pediatric age group. It may affect the orbits, lacrimal and salivary glands, pancreas, kidneys, peritoneum and other organs. Lung and pleura are not commonly reported in IgG4-RD. We here present a rare case of pediatric IgG4-RD with rare involvement of pericardium, pleura and lungs. CASE PRESENTATION: A 13-year-old girl presented with intrathoracic IgG4-RD with pleuropericardial involvement...