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Cavitating Lung Disease: A Novel Presentation of IgG4-Related Disease.

Jinnur PK, Yi ES, Ryu JH, Iyer VN - Am J Case Rep (2015)

Bottom Line: Follow-up 1 year later shows stable pulmonary function with complete resolution of the cavitary lesions.Our patient had an excellent response to immunosuppression.An increased awareness of IgG4-related disease and its myriad of manifestations is very important for pulmonologists.

View Article: PubMed Central - PubMed

Affiliation: Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA.

ABSTRACT

Background: Immunoglobulin (Ig) G4-related disease, previously referred to as IgG4-related sclerosing disease or hyper-IgG4 disease, may occur in the lung, involving alveolar parenchyma, airways, and pleura. Various pulmonary manifestations of IgG4-related disease have been reported, but to the best of our knowledge a cavitating lung disease has not been reported previously.

Case report: We describe a 60-year-old man who presented with hemoptysis and cavitating lung disease with clinical, laboratory, and histopathologic findings compatible with IgG4-related disease. Other potential causes of cavitation were excluded. Treatment was initiated with oral prednisone and subsequently mycophenolate mofetil was added. Follow-up 1 year later shows stable pulmonary function with complete resolution of the cavitary lesions.

Conclusions: We present a case of cavitating lung disease as a previously unreported manifestation of IgG4-related disease. Our patient had an excellent response to immunosuppression. An increased awareness of IgG4-related disease and its myriad of manifestations is very important for pulmonologists.

No MeSH data available.


Related in: MedlinePlus

Follow-up CT chest 1 year later showing complete resolution of previously noted cavitary lesions in the left upper and lower lobes, with residual scarring.
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f4-amjcaserep-16-478: Follow-up CT chest 1 year later showing complete resolution of previously noted cavitary lesions in the left upper and lower lobes, with residual scarring.

Mentions: Although the presence of neutrophilic aggregates has been noted in pulmonary IgG4-related lung disease (IgG4-RLD), a cavitary lesion has not been documented as a feature of IgG4-RLD. We believe that this case could be a possible cavitary IgG4-RLD in light of his other manifestations, including the parotid gland enlargement elevated serum IgG4 level, and the lack of any viable alternative diagnoses. While the histopathologic diagnosis was challenging due to the presence of cavitary lesion, the remaining histopathologic findings with increased IgG4+ plasma cells were acceptable for IgG4-RLD. There was no evidence of extrapulmonary IgG4-related disease other than parotid gland enlargement. Treatment was initiated with oral prednisone 60 mg/day, and subsequently mycophenolate mofetil (MMF) was added with slow tapering of prednisone over the following 2 months. Follow-up 1 year later while on 2 gm/day of MMF and a tapering dose of prednisone at 5 mg per day showed stable pulmonary function. Follow-up CT chest (Figure 4) 1 year later revealed complete resolution of the cavitary lesions, with residual scarring. Parotid gland swelling also resolved. Serum IgG4 levels normalized 6 weeks after initiation of prednisone.


Cavitating Lung Disease: A Novel Presentation of IgG4-Related Disease.

Jinnur PK, Yi ES, Ryu JH, Iyer VN - Am J Case Rep (2015)

Follow-up CT chest 1 year later showing complete resolution of previously noted cavitary lesions in the left upper and lower lobes, with residual scarring.
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4514329&req=5

f4-amjcaserep-16-478: Follow-up CT chest 1 year later showing complete resolution of previously noted cavitary lesions in the left upper and lower lobes, with residual scarring.
Mentions: Although the presence of neutrophilic aggregates has been noted in pulmonary IgG4-related lung disease (IgG4-RLD), a cavitary lesion has not been documented as a feature of IgG4-RLD. We believe that this case could be a possible cavitary IgG4-RLD in light of his other manifestations, including the parotid gland enlargement elevated serum IgG4 level, and the lack of any viable alternative diagnoses. While the histopathologic diagnosis was challenging due to the presence of cavitary lesion, the remaining histopathologic findings with increased IgG4+ plasma cells were acceptable for IgG4-RLD. There was no evidence of extrapulmonary IgG4-related disease other than parotid gland enlargement. Treatment was initiated with oral prednisone 60 mg/day, and subsequently mycophenolate mofetil (MMF) was added with slow tapering of prednisone over the following 2 months. Follow-up 1 year later while on 2 gm/day of MMF and a tapering dose of prednisone at 5 mg per day showed stable pulmonary function. Follow-up CT chest (Figure 4) 1 year later revealed complete resolution of the cavitary lesions, with residual scarring. Parotid gland swelling also resolved. Serum IgG4 levels normalized 6 weeks after initiation of prednisone.

Bottom Line: Follow-up 1 year later shows stable pulmonary function with complete resolution of the cavitary lesions.Our patient had an excellent response to immunosuppression.An increased awareness of IgG4-related disease and its myriad of manifestations is very important for pulmonologists.

View Article: PubMed Central - PubMed

Affiliation: Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA.

ABSTRACT

Background: Immunoglobulin (Ig) G4-related disease, previously referred to as IgG4-related sclerosing disease or hyper-IgG4 disease, may occur in the lung, involving alveolar parenchyma, airways, and pleura. Various pulmonary manifestations of IgG4-related disease have been reported, but to the best of our knowledge a cavitating lung disease has not been reported previously.

Case report: We describe a 60-year-old man who presented with hemoptysis and cavitating lung disease with clinical, laboratory, and histopathologic findings compatible with IgG4-related disease. Other potential causes of cavitation were excluded. Treatment was initiated with oral prednisone and subsequently mycophenolate mofetil was added. Follow-up 1 year later shows stable pulmonary function with complete resolution of the cavitary lesions.

Conclusions: We present a case of cavitating lung disease as a previously unreported manifestation of IgG4-related disease. Our patient had an excellent response to immunosuppression. An increased awareness of IgG4-related disease and its myriad of manifestations is very important for pulmonologists.

No MeSH data available.


Related in: MedlinePlus