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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.


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CT showed collapse and consolidation in the right upper lobe with adjacent areas of branching opacities
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Figure 1: CT showed collapse and consolidation in the right upper lobe with adjacent areas of branching opacities

Mentions: On evaluation, his chest x-ray revealed nonhomogenous consolidation of the right upper zone, with an air bronchogram. His blood counts and routine biochemistries were normal. His Mantoux test and sputum smear and culture for Mycobacterium tuberculosis bacilli were negative. We proceeded to do computed tomography (CT) of the thorax, which revealed collapse and consolidation in the right upper lobe with adjacent areas of branching opacities [Figure 1]. With a wide range of differentials in mind, a battery of blood tests were further ordered. These revealed a serum IgG4 of 4264 mg/L (normal range 100 - 1400 mg/L). Serum antineutrophilic antibodies were not detected. Rheumatoid factor (RA) and C-reactive protein (CRP) were within normal limits. Hence, he was considered to have pulmonary IgG4 disease with differentials, such as, sarcoidosis, bronchogenic carcinoma, lymphoma, and Castleman disease.[2]


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)

CT showed collapse and consolidation in the right upper lobe with adjacent areas of branching opacities
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 1: CT showed collapse and consolidation in the right upper lobe with adjacent areas of branching opacities
Mentions: On evaluation, his chest x-ray revealed nonhomogenous consolidation of the right upper zone, with an air bronchogram. His blood counts and routine biochemistries were normal. His Mantoux test and sputum smear and culture for Mycobacterium tuberculosis bacilli were negative. We proceeded to do computed tomography (CT) of the thorax, which revealed collapse and consolidation in the right upper lobe with adjacent areas of branching opacities [Figure 1]. With a wide range of differentials in mind, a battery of blood tests were further ordered. These revealed a serum IgG4 of 4264 mg/L (normal range 100 - 1400 mg/L). Serum antineutrophilic antibodies were not detected. Rheumatoid factor (RA) and C-reactive protein (CRP) were within normal limits. Hence, he was considered to have pulmonary IgG4 disease with differentials, such as, sarcoidosis, bronchogenic carcinoma, lymphoma, and Castleman disease.[2]

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.


Related in: MedlinePlus