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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 scan shows the resolved lesion
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Figure 4: CT scan shows the resolved lesion

Mentions: A review after six months from the start of treatment, revealed a recurrence of the consolidation [Figure 3] with a simultaneous increase in the IgG4 levels to 3352 mg/l. He was diagnosed with recurrence of pulmonary IgG4 disease. The prednisolone dose was hiked to 0.75 mg/kg body weight a day and a follow-up CT scan after three months showed near complete resolution of the consolidation [Figure 4] and the IgG4 level decreased to 1190 mg/l. The pathological features proposed for the diagnosis of IgG4 disease include, the presence of marked lymphocyte plasma cell infiltration and fibrosis, with IgG4-positive plasma cell infiltrates, that is, aIgG4/IgG ratio of >40%, and the occurrence of 10 IgG4-positive cells per HPF.[1] The characteristic CT features of IgG4 -related pulmonary lesions include, (a) solitary nodular opacities, (b) Round, ground glass–like opacities with relatively discrete margins, (c) honeycomb lung opacities, and (d)bronchovascular type.


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 scan shows the resolved lesion
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 4: CT scan shows the resolved lesion
Mentions: A review after six months from the start of treatment, revealed a recurrence of the consolidation [Figure 3] with a simultaneous increase in the IgG4 levels to 3352 mg/l. He was diagnosed with recurrence of pulmonary IgG4 disease. The prednisolone dose was hiked to 0.75 mg/kg body weight a day and a follow-up CT scan after three months showed near complete resolution of the consolidation [Figure 4] and the IgG4 level decreased to 1190 mg/l. The pathological features proposed for the diagnosis of IgG4 disease include, the presence of marked lymphocyte plasma cell infiltration and fibrosis, with IgG4-positive plasma cell infiltrates, that is, aIgG4/IgG ratio of >40%, and the occurrence of 10 IgG4-positive cells per HPF.[1] The characteristic CT features of IgG4 -related pulmonary lesions include, (a) solitary nodular opacities, (b) Round, ground glass–like opacities with relatively discrete margins, (c) honeycomb lung opacities, and (d)bronchovascular type.

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