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Immunoglobulin G4-related disease with features of Mikulicz's disease and autoimmune pancreatitis which firstly presented as asymptomatic lymphadenopathy: a case report.

Wu Y, Xu ZR, Zhou WJ, Yang YM - Chin. Med. J. (2015)

View Article: PubMed Central - PubMed

Affiliation: Department of Geriatrics, First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, Zhejiang 310013, China.

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To the Editor: Mikulicz's disease (MD), also known as benign lymphoepithelial lesion, refers to idiopathic, bilateral, painless, and symmetrical swelling of the lacrimal, parotid, and submandibular glands... The complications of MD include autoimmune pancreatitis, retroperitoneal fibrosis, tubulointerstitial nephritis, autoimmune hypophysitis, and Riedel's thyroiditis, all of which show elevated serum immunoglobulin G4 (IgG4) levels and prominent infiltration of IgG4-positive plasmacytes... An 82-year-old man was admitted to our hospital on April 16, 2012 because of anorexia, anergy, emaciation, and painless lumps in postaurem... Five years ago, he found a painless lump (about 2.3 cm × 1.2 cm) both in his left and right postaurem and the left one was surgically excised after 1 year... Subsequent positron emission tomography and computed tomography (PET-CT) scan images showed abnormal 18-fluorodeoxyglucose (FDG) uptake in the bilateral submandibular glands and parotid glands... The maximum standardized uptake value (SUV) of the submandibular glands was 11.16 and of the parotid glands was 10.86... These clinical, laboratory, and imaging findings indicated the possibility of IgG4-RD involving the lymph nodes, submandibular glands, and pancreas, according to the comprehensive diagnostic criteria for IgG4-RD... Treatment guidelines for IgG4-RD have not been developed... Corticosteroid therapy has been regarded as an effective treatment... Himi et al., initiated treatment with prednisolone at 30–40 mg/day against MD without encountering organ failure, and suggested that it is necessary to continue administering at 5–10 mg/day or to combine it with an immunosuppressant... Taking into account the advanced age of our patient, we decreased the dose of corticosteroid, and he was successfully treated... However, the optimal initial doses of steroids, tapering procedures, and maintenance doses are still controversial... Further investigation is necessary to establish the most effective therapy... Here, we have described a typical case of IgG4-RD, previously mis-diagnosed... This time he was successfully treated.

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Biopsy specimen of right submandibular gland. (a) Salivary glands are atrophic and cirrhotic, and fibrous tissues and myofibroblasts are proliferating. Interstitial tissues are infiltrated with large numbers of lymphocytes and plasmacytes (H and E, ×100); (b) Immunostaining indicating diffuse infiltration of immunoglobulin G4 (IgG4) (+) plasma cells. The ratio of IgG4/IgG positive cells was >50% and there were >10 IgG4-positive plasma cells/high power field (IgG4 immunostaining, Original magnification, ×200).
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Figure 2: Biopsy specimen of right submandibular gland. (a) Salivary glands are atrophic and cirrhotic, and fibrous tissues and myofibroblasts are proliferating. Interstitial tissues are infiltrated with large numbers of lymphocytes and plasmacytes (H and E, ×100); (b) Immunostaining indicating diffuse infiltration of immunoglobulin G4 (IgG4) (+) plasma cells. The ratio of IgG4/IgG positive cells was >50% and there were >10 IgG4-positive plasma cells/high power field (IgG4 immunostaining, Original magnification, ×200).

Mentions: These clinical, laboratory, and imaging findings indicated the possibility of IgG4-RD involving the lymph nodes, submandibular glands, and pancreas, according to the comprehensive diagnostic criteria for IgG4-RD.[4] A right submandibular gland biopsy was performed in May 2012 for confirmation. The pathological report stated that the salivary glands were atrophic and cirrhotic, and fibrous tissues and myofibroblasts were proliferating. Interstitial tissues were infiltrated with large numbers of lymphocytes and plasmacytes. Immunostaining indicated diffuse infiltration of IgG4 (+) plasma cells. The ratio of IgG4/IgG positive cells was >50% and there were >10 IgG4-positive plasma cells per high power field (HPF) [Figure 2].


Immunoglobulin G4-related disease with features of Mikulicz's disease and autoimmune pancreatitis which firstly presented as asymptomatic lymphadenopathy: a case report.

Wu Y, Xu ZR, Zhou WJ, Yang YM - Chin. Med. J. (2015)

Biopsy specimen of right submandibular gland. (a) Salivary glands are atrophic and cirrhotic, and fibrous tissues and myofibroblasts are proliferating. Interstitial tissues are infiltrated with large numbers of lymphocytes and plasmacytes (H and E, ×100); (b) Immunostaining indicating diffuse infiltration of immunoglobulin G4 (IgG4) (+) plasma cells. The ratio of IgG4/IgG positive cells was >50% and there were >10 IgG4-positive plasma cells/high power field (IgG4 immunostaining, Original magnification, ×200).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4834790&req=5

Figure 2: Biopsy specimen of right submandibular gland. (a) Salivary glands are atrophic and cirrhotic, and fibrous tissues and myofibroblasts are proliferating. Interstitial tissues are infiltrated with large numbers of lymphocytes and plasmacytes (H and E, ×100); (b) Immunostaining indicating diffuse infiltration of immunoglobulin G4 (IgG4) (+) plasma cells. The ratio of IgG4/IgG positive cells was >50% and there were >10 IgG4-positive plasma cells/high power field (IgG4 immunostaining, Original magnification, ×200).
Mentions: These clinical, laboratory, and imaging findings indicated the possibility of IgG4-RD involving the lymph nodes, submandibular glands, and pancreas, according to the comprehensive diagnostic criteria for IgG4-RD.[4] A right submandibular gland biopsy was performed in May 2012 for confirmation. The pathological report stated that the salivary glands were atrophic and cirrhotic, and fibrous tissues and myofibroblasts were proliferating. Interstitial tissues were infiltrated with large numbers of lymphocytes and plasmacytes. Immunostaining indicated diffuse infiltration of IgG4 (+) plasma cells. The ratio of IgG4/IgG positive cells was >50% and there were >10 IgG4-positive plasma cells per high power field (HPF) [Figure 2].

View Article: PubMed Central - PubMed

Affiliation: Department of Geriatrics, First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, Zhejiang 310013, China.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

To the Editor: Mikulicz's disease (MD), also known as benign lymphoepithelial lesion, refers to idiopathic, bilateral, painless, and symmetrical swelling of the lacrimal, parotid, and submandibular glands... The complications of MD include autoimmune pancreatitis, retroperitoneal fibrosis, tubulointerstitial nephritis, autoimmune hypophysitis, and Riedel's thyroiditis, all of which show elevated serum immunoglobulin G4 (IgG4) levels and prominent infiltration of IgG4-positive plasmacytes... An 82-year-old man was admitted to our hospital on April 16, 2012 because of anorexia, anergy, emaciation, and painless lumps in postaurem... Five years ago, he found a painless lump (about 2.3 cm × 1.2 cm) both in his left and right postaurem and the left one was surgically excised after 1 year... Subsequent positron emission tomography and computed tomography (PET-CT) scan images showed abnormal 18-fluorodeoxyglucose (FDG) uptake in the bilateral submandibular glands and parotid glands... The maximum standardized uptake value (SUV) of the submandibular glands was 11.16 and of the parotid glands was 10.86... These clinical, laboratory, and imaging findings indicated the possibility of IgG4-RD involving the lymph nodes, submandibular glands, and pancreas, according to the comprehensive diagnostic criteria for IgG4-RD... Treatment guidelines for IgG4-RD have not been developed... Corticosteroid therapy has been regarded as an effective treatment... Himi et al., initiated treatment with prednisolone at 30–40 mg/day against MD without encountering organ failure, and suggested that it is necessary to continue administering at 5–10 mg/day or to combine it with an immunosuppressant... Taking into account the advanced age of our patient, we decreased the dose of corticosteroid, and he was successfully treated... However, the optimal initial doses of steroids, tapering procedures, and maintenance doses are still controversial... Further investigation is necessary to establish the most effective therapy... Here, we have described a typical case of IgG4-RD, previously mis-diagnosed... This time he was successfully treated.

Show MeSH
Related in: MedlinePlus