IgG4-related lung disease presenting as interstitial lung disease with bronchiolitis

Chen, Chiu-Fan; Chu, Kuo-An; Tseng, Yen-Chiang; Wu, Chang-Che; Lai, Ruay-Sheng

doi:10.1097/md.0000000000009140

Cited by 13 publications

(9 citation statements)

References 12 publications

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“…IgG4‐related diseases encompass a spectrum of disorders with raised serum IgG4 levels and substantial infiltration by IgG4 positive plasma cells that involve different body organs either as isolated or multisystemic lesions 10 . Lung manifestations include inflammatory pseudotumor, interstitial pneumonitis, organizing pneumonia, bronchiectasis, bronchiolitis and lymphomatoid granulomatosis 5‐8,10 . Patients with organ involvement or damage consistent with IgG4‐related respiratory disease, IgG4 levels of >135 mg/dL in serum, histopathology showing marked lymphoplasmacytic infiltration, storiform fibrosis, obliterative phlebitis, eosinophilic infiltrates, and IgG4/IgG ratio of >40% are diagnosed with IgG4‐related respiratory disease.…”

Section: Discussionmentioning

confidence: 99%

“…Patients with organ involvement or damage consistent with IgG4‐related respiratory disease, IgG4 levels of >135 mg/dL in serum, histopathology showing marked lymphoplasmacytic infiltration, storiform fibrosis, obliterative phlebitis, eosinophilic infiltrates, and IgG4/IgG ratio of >40% are diagnosed with IgG4‐related respiratory disease. Although, an increase of IgG4 positive plasma cells is helpful for suspecting the diagnosis, elevated serum IgG4 and plasma cells may also be seen in bronchiectasis, asthma, idiopathic pulmonary fibrosis, emphysema, hypersensitivity pneumonitis, primary sclerosing cholangitis, inflammatory bowel disease, Hashimoto's thyroiditis, and in 5% of the normal population 5‐11 . The accuracy of serum IgG4 levels of >135‐144 mg/dL showed a sensitivity of 87% and specificity of 83% for diagnosing IgG4‐related respiratory disease 11 .…”

Section: Discussionmentioning

confidence: 99%

“…They include extrinsic allergic alveolitis, organizing pneumonia, and chronic eosinophilic pneumonia. Recently, reports of IgG4 plasma cell‐related diseases involving the lungs have been described mostly in tissue specimens 5‐8 . There are few reports of this diagnosis made in cytologic specimens.…”

Section: Introductionmentioning

confidence: 99%

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Plasma cell‐rich bronchoalveolar lavage fluid: Could be a first clue to IgG4 plasma cell‐related lung disease

AbdullGaffar

Abdelkareem

2021

Diagnostic Cytopathology

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Plasma cells are usually absent in normal bronchoalveolar lavage (BAL) fluids. Frequent plasma cells in BAL are always pathologically significant. They raise a rather narrow differential diagnosis, predominantly extrinsic allergic alveolitis and few other interstitial lung diseases. BAL is a key diagnostic procedure for certain lung diseases. Because plasma cells are uncommon in BAL fluids and usually associated with limited conditions compared to other more common interstitial lung diseases, the focus of cell count differentials in BAL fluids was on other mononuclear and granulocytic inflammatory cells. Recently, cases of IgG4 positive plasma cell‐related lung diseases have been reported in tissue specimens. This may raise the importance of cytologically recognizing plasma cells in BAL fluids. We report a case of a 16‐year‐old boy with clinical and radiologic features of diffuse interstitial lung disease. His BAL cytology showed frequent plasma cells. Endobronchial biopsy showed substantial infiltration by plasma cells confirmed by immunohistochemistry. After excluding other lung diseases, the possibility of IgG4 plasma cell‐related disease was raised. Blood tests showed elevated serum levels of IgG4. This case emphasizes the importance of including of IgG4 plasma cell‐related lung disease as a possible differential diagnosis. This should prompt proper serologic tests for confirmation and appropriate management. With increasing reports of IgG4‐related lung disease, cytopathologists should be aware of this possibility when examining BAL fluids. Measurement of IgG levels in BAL fluids and plasma cell immunophenotyping might be promising tools.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Plasma cell‐rich bronchoalveolar lavage fluid: Could be a first clue to IgG4 plasma cell‐related lung disease

AbdullGaffar

Abdelkareem

2021

Diagnostic Cytopathology

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“…IgG4-RD is a novel multi-organ inflammatory disorder characterized by the subacute development of a mass-like swelling or enlargement of an organ, increased serum levels of IgG4, infiltration of IgG4-positive lymphocytes, and storiform fibrosis of tissue (3,7,8). It usually occurs in middle-aged Asian males and responds well to corticosteroid treatment (9).…”

Section: Discussionmentioning

confidence: 99%

Immunoglobulin G4-Related Disease Unilaterally Involving the Pulmonary Interstitium and Pleura: A Case Report

et al. 2018

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Immunoglobulin G4-related disease is a novel inflammatory entity involving multiple organs of the body. In the lungs, it is accompanied by infiltration of IgG4-positive lymphocytes and increased serum levels of IgG4. Previous studies have reported radiological findings of IgG4-related lung disease in the pulmonary parenchyma; however, there are few case reports of pleural involvement. Here, we report the case of a 66-year-old man with IgG4-related disease unilaterally involving the lung interstitium and pleura. Thoracic computed tomography images demonstrated pleural effusion and thickening resembling a primary or secondary pleural malignancy. The diagnosis of IgG4-related disease was confirmed by video-assisted-thoracoscopic biopsy. This rare disease should not be overlooked in patients with radiological findings that are suggestive of pleural malignancies.

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“…It shows some mediastinal lymph node enlargement and also shows multiple mosaic attenuation. 2 . It may involve multiple organs including lungs, mediastinum , liver and pancreas.…”

Section: Introductionmentioning

confidence: 99%

IgG4 Related Lung Disorder -A Review

2020

IJFMT

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IgG4 disorder is an autoimmune disorder. Its manifestation in the lung causes fibroinflammatory disorder. It is one of the chronic inflammatory disorders. IgG4 is the subclass of IgG. This review is analysed based on the IgG4 related disorder. Nearly 50 articles were reviewed and this study was conducted for a period of nearly 15 years.The IgG4 related disorder is a very rare disorder in the world. Moreover, It is an autoimmune disorder like Systemic lupus erythematosus. It is related to systemic disorder and chronic inflammatory disorder IgG4 varies high degree of fibrosis and it is prompted by the response of steroid therapy.It is the most common form of immunoglobulin. Ig G also known as secretory immunoglobulin.Ig G4 is a subclass of IgG comprising only 4% in gamma immunoglobulins . IgG4 does not bind to polysaccharides. Increased levels of IgG4 in serum usually found in patients suffering sclerosing pancreatitis, cholangitis and interstitial pneumonia caused by the infiltration of IgG4 positive plasma cells. IgG4 has a molecular weight of 159. IgG4 related disorder recognised as one of the rare autoimmune disorders which can affect multiple organs and cause organ failure sometimes. It involves the complement system and interacts with the antibody. Generally IgG4 related disorder is recognised as one of the most rare autoimmune disorders which can affect multiple organ failure sometimes. It involves complement system and interact with antibody. Generally, IgG4 related disorder most commonly present in in beekeper, animal laboratory workers and individuals who have undergone allergen immunotherapy possess high serum level of IgG4.it is one of the acquired Type II hypersensitivity disorder. This review mainly aims to know the reason behind the clinical lung disorder.

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IgG4-related lung disease presenting as interstitial lung disease with bronchiolitis

Cited by 13 publications

References 12 publications

Plasma cell‐rich bronchoalveolar lavage fluid: Could be a first clue to IgG4 plasma cell‐related lung disease

Plasma cell‐rich bronchoalveolar lavage fluid: Could be a first clue to IgG4 plasma cell‐related lung disease

Immunoglobulin G4-Related Disease Unilaterally Involving the Pulmonary Interstitium and Pleura: A Case Report

IgG4 Related Lung Disorder -A Review

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