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Isolated pulmonary manifestation of IgG4 disease with response to steroids and relapse: A rare case report.

Singh RK, Isaac TJ, Thangakunam B, Mathews N, Korula A - Lung India (2015 Nov-Dec)

View Article: PubMed Central - PubMed

Affiliation: Department of Pulmonary Medicine, Christian Medical College, Vellore, Tamil Nadu, India. E-mail: ranjitcmc.2011@gmail.com.

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Sir, IgG4-related disease is currently considered as a disease of unknown etiology, with commonly shared features that include an elevated serum concentration of IgG4-pronounced lymphocytes and IgG4-positive plasma cell infiltrates and fibrosis with consequent swelling of the involved organs, as well as nodulations and hyperplastic lesions... There were dense infiltrates of plasma cells and IgG4 immunostaining showed positive cytoplasm staining in most of the plasma cells... Quantifying, 60–80 IgG4 positive plasma cells per HPF were found... A dose of 0.5 mg/kg/body of prednisolone was prescribed for a month, with subsequent gradual tapering, at the rate of 4 mg per month... A follow-up CT of the chest after four months showed that the consolidation had reduced in size, with areas of scarring, and his serum IgG4 levels dropped significantly to 2881mg/l... The steroid dose was further reduced... Castleman disease is characterized by lymph node enlargement because of hyperplasia of the abnormal lymphoid follicles and paracortical lymphocytic hyaline vascular stroma or plasmacytosis... IgG4 diseases respond well to glucocoticosteriods within several weeks, especially with symptomatic improvement, reduction in the size of the masses, and often a decrease in the serum levels of IgG4... The solitary manifestation of an IgG4-related pulmonary lesion may show findings similar to those of sarcoidosis and Castleman disease... Therefore, differentiation between these conditions, correlation with serum IgG4 levels, and the histopathology of lung biopsy specimens is necessary... In conclusion, IgG4 disease has a myriad of pulmonary manifestations... Histopathology with IgG4 imunostaining and measuring serum levels of IgG4 help in clinching the diagnosis... The correlation of clinicoradiological and pathological features is of extreme importance for the diagnosis of IgG4-related lung disease.

No MeSH data available.


The histology photographed revealed a dense lymphoplasmacytic infiltrate with entrapped bronchial glands (arrow). The IgG immunostain showed dense infiltrates of plasma cells. The IgG4 immunostain showed positive cytoplasmic staining in most of the plasma cells
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Figure 2: The histology photographed revealed a dense lymphoplasmacytic infiltrate with entrapped bronchial glands (arrow). The IgG immunostain showed dense infiltrates of plasma cells. The IgG4 immunostain showed positive cytoplasmic staining in most of the plasma cells

Mentions: A CT-guided biopsy was performed to determine the etiology, which revealed a dense lymphoplasmacytic infiltrate, with entrapped bronchial glands (arrow) [Figure 2]. There were dense infiltrates of plasma cells and IgG4 immunostaining showed positive cytoplasm staining in most of the plasma cells. Quantifying, 60–80 IgG4 positive plasma cells per HPF were found. The ratio of IgG: IgG4 positive plasma cells were 60–80%. This confirmed the suspicion of IgG4 disease.


Isolated pulmonary manifestation of IgG4 disease with response to steroids and relapse: A rare case report.

Singh RK, Isaac TJ, Thangakunam B, Mathews N, Korula A - Lung India (2015 Nov-Dec)

The histology photographed revealed a dense lymphoplasmacytic infiltrate with entrapped bronchial glands (arrow). The IgG immunostain showed dense infiltrates of plasma cells. The IgG4 immunostain showed positive cytoplasmic staining in most of the plasma cells
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4663883&req=5

Figure 2: The histology photographed revealed a dense lymphoplasmacytic infiltrate with entrapped bronchial glands (arrow). The IgG immunostain showed dense infiltrates of plasma cells. The IgG4 immunostain showed positive cytoplasmic staining in most of the plasma cells
Mentions: A CT-guided biopsy was performed to determine the etiology, which revealed a dense lymphoplasmacytic infiltrate, with entrapped bronchial glands (arrow) [Figure 2]. There were dense infiltrates of plasma cells and IgG4 immunostaining showed positive cytoplasm staining in most of the plasma cells. Quantifying, 60–80 IgG4 positive plasma cells per HPF were found. The ratio of IgG: IgG4 positive plasma cells were 60–80%. This confirmed the suspicion of IgG4 disease.

View Article: PubMed Central - PubMed

Affiliation: Department of Pulmonary Medicine, Christian Medical College, Vellore, Tamil Nadu, India. E-mail: ranjitcmc.2011@gmail.com.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Sir, IgG4-related disease is currently considered as a disease of unknown etiology, with commonly shared features that include an elevated serum concentration of IgG4-pronounced lymphocytes and IgG4-positive plasma cell infiltrates and fibrosis with consequent swelling of the involved organs, as well as nodulations and hyperplastic lesions... There were dense infiltrates of plasma cells and IgG4 immunostaining showed positive cytoplasm staining in most of the plasma cells... Quantifying, 60–80 IgG4 positive plasma cells per HPF were found... A dose of 0.5 mg/kg/body of prednisolone was prescribed for a month, with subsequent gradual tapering, at the rate of 4 mg per month... A follow-up CT of the chest after four months showed that the consolidation had reduced in size, with areas of scarring, and his serum IgG4 levels dropped significantly to 2881mg/l... The steroid dose was further reduced... Castleman disease is characterized by lymph node enlargement because of hyperplasia of the abnormal lymphoid follicles and paracortical lymphocytic hyaline vascular stroma or plasmacytosis... IgG4 diseases respond well to glucocoticosteriods within several weeks, especially with symptomatic improvement, reduction in the size of the masses, and often a decrease in the serum levels of IgG4... The solitary manifestation of an IgG4-related pulmonary lesion may show findings similar to those of sarcoidosis and Castleman disease... Therefore, differentiation between these conditions, correlation with serum IgG4 levels, and the histopathology of lung biopsy specimens is necessary... In conclusion, IgG4 disease has a myriad of pulmonary manifestations... Histopathology with IgG4 imunostaining and measuring serum levels of IgG4 help in clinching the diagnosis... The correlation of clinicoradiological and pathological features is of extreme importance for the diagnosis of IgG4-related lung disease.

No MeSH data available.