IgG4‐related systemic disease and lymphoplasmacytic aortitis

Stone, John H.; Khosroshahi, Arezou; Hilgenberg, Alan D.; Spooner, Amy E.; Isselbacher, Eric M.; Stone, James R.

doi:10.1002/art.24798

Cited by 169 publications

(109 citation statements)

References 35 publications

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“…8,10,[11][12][13][14] The disease presented with uniform clinicopathology, namely asymptomatic localized submandibular lymphadenopathy and progression to extranodal lesions, particularly the lacrimal and submandibular glands. Patients were predominantly middle-aged to older males, and about half of those examined had concomitant allergic disease.…”

Section: Discussionmentioning

confidence: 99%

“…These clinicopathological findings of IgG4 þ progressively transformed germinal centers are compatible with IgG4-related disease. 8,10,[11][12][13][14] IgG4-related disease frequently involves the lacrimal glands, submandibular glands, pancreas, hepatobiliary tract and lymph nodes. [7][8][9][10][11][12][13][14] Nevertheless, virtually any organ can be affected, including the lungs, mediastinum, skin, retroperitoneum, aorta, kidneys and prostate.…”

Section: Discussionmentioning

confidence: 99%

“…8,10,[11][12][13][14] IgG4-related disease frequently involves the lacrimal glands, submandibular glands, pancreas, hepatobiliary tract and lymph nodes. [7][8][9][10][11][12][13][14] Nevertheless, virtually any organ can be affected, including the lungs, mediastinum, skin, retroperitoneum, aorta, kidneys and prostate. [7][8][9][10][11][12][13][14] The general condition of patients at presentation is usually good, with no fever or constitutional symptoms.…”

Section: Discussionmentioning

confidence: 99%

“…[7][8][9][10][11][12][13][14] Nevertheless, virtually any organ can be affected, including the lungs, mediastinum, skin, retroperitoneum, aorta, kidneys and prostate. [7][8][9][10][11][12][13][14] The general condition of patients at presentation is usually good, with no fever or constitutional symptoms. Common laboratory findings include increased serum IgG4 and IgE levels, whereas lactate dehydrogenase level remains unchanged.…”

Section: Discussionmentioning

confidence: 99%

“…[2][3][4][5][6] IgG4-related disease is a recently recognized syndrome characterized by mass-forming lesions with lymphoplasmacytic infiltration, accumulation of IgG4 þ plasma cells in affected tissues and increased serum IgG4 levels. [7][8][9][10][11][12][13][14] IgG4-related disease generally involves either localized or systemic lymph nodes, 8,13,14 and five histological subtypes of IgG4-related lymphadenopathy have been recognized. 8 In 2009, we were the first to report patients with IgG4-related disease in progressively transformed germinal centers of the lymph nodes (progressively transformed germinal centers-type IgG4-related lymphadenopathy).…”

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confidence: 99%

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Association between IgG4-related disease and progressively transformed germinal centers of lymph nodes

et al. 2012

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Progressively transformed germinal centers is a benign condition of unknown pathogenesis characterized by a distinctive variant form of reactive follicular hyperplasia in lymph nodes. We recently reported Ig G4-related disease in progressively transformed germinal centers. However, no large case series has been reported and clinicopathologic findings remain unclear. Here, we report 40 Japanese patients (28 men, 12 women; median age, 56 years) with progressively transformed germinal centers of the lymph nodes who fulfilled the histological diagnostic criteria for IgG4-related disease (IgG4 þ progressively transformed germinal centers), with asymptomatic localized lymphadenopathy involving the submandibular nodes in 24, submandibular and cervical nodes in 14, cervical nodes only in 1, and cervical and supraclavicular nodes in 1. In all, 16 (52%) of 31 examined patients had allergic disease. Histologically, the lymph nodes demonstrated uniform histological findings, namely marked follicular hyperplasia with progressively transformed germinal centers, and localization of the majority of IgG4 þ plasma cells in the germinal centers. Serum IgG4, serum IgE and peripheral blood eosinophils were elevated in 87%, 92% and 53% of examined patients, respectively. Eighteen patients subsequently developed extranodal lesions (including five who developed systemic disease), which on histological examination were consistent with IgG4-related disease. IgG4 þ progressively transformed germinal centers presents with uniform clinicopathological features of asymptomatic localized submandibular lymphadenopathy, which persists and/or relapses, and sometimes progresses to extranodal lesions or systemic disease. Nine patients were administered steroid therapy when the lesions progressed, to which all

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

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Association between IgG4-related disease and progressively transformed germinal centers of lymph nodes

et al. 2012

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IgG4‐related systemic disease accounts for a significant proportion of thoracic lymphoplasmacytic aortitis cases

Stone¹,

Khosroshahi²,

Stone³

2010

Arthritis Care & Research

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Objective. IgG4-related systemic disease, a disorder recognized only recently, can cause lymphoplasmacytic inflammation in the thoracic aorta. The percentage of cases caused by IgG4-related systemic disease is not known. We aimed to determine the percentage of noninfectious thoracic aortitis cases that are associated with IgG4-related systemic disease and to establish pathologic criteria for identifying involvement of the thoracic aorta by this disorder. Methods. We searched our Pathology Service database to identify patients with noninfectious thoracic aortitis who underwent resection over a 5-year time span. The histologic features of these cases were reviewed. All cases of lymphoplasmacytic aortitis and representative cases of giant cell aortitis and atherosclerosis were stained by immunohistochemistry for IgG4 and for the plasma cell marker CD138. We determined the fraction of plasma cells that stained for IgG4. Results. Of 638 resected thoracic aortas, 33 (5.2%) contained noninfectious aortitis. Four of these cases (12% of all patients with noninfectious aortitis) had histologic features of lymphoplasmacytic aortitis. Three of those 4 cases (9% of the noninfectious aortitis cases) demonstrated pathologic involvement by

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A 55‐Year‐Old Man With Periorbital and Inguinal Masses, Pericarditis, and Pleuritis

Gupta

Frater

et al. 2017

Arthritis Care & Research

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A 55-year-old male was referred to the rheumatology clinic for evaluation of recurrent inguinal and periorbital masses. History of the present illnessA 55-year-old white man with ulcerative colitis in remission and coronary artery disease developed an indurated 5-cm right inguinal subcutaneous mass approximately 6 years prior to presentation. An excisional biopsy demonstrated lymphoid follicular hyperplasia. Three years later, the right inguinal mass reappeared. Repeat surgical excision revealed a xanthogranulomatous infiltrate with necrobiosis, cholesterol clefts, and Touton giant cells (large multinucleated cells with a ring of nuclei surrounded by foamy cytoplasm). Gomori methenamine silver and Fite stains were negative for fungal or mycobacterial organisms. Despite removal, the mass reappeared within 4 months and slowly but progressively increased in size, accompanied by hyperpigmentation and a yellow discoloration of the overlying skin. One year prior to presentation, he developed shortness of breath and was found to have pericarditis complicated by pericardial effusion and tamponade for which he underwent a pericardiocentesis and pericardial window. Cytology of the fluid was negative for malignant cells. A pericardial biopsy demonstrated a thickened pericardial wall with florid chronic inflammation, fibrosis, and numerous plasma cells. In situ hybridization studies showed a kappa restricted monoclonal plasma cell population. Around that time, he also developed a tight band-like mass at the base of his penis. The right inguinal mass continued to increase in size, and ulceration of the overlying skin developed. Nine months prior to presentation, he noted a left periorbital 1-cm mass that increased in size, leading to ptosis and decreased peripheral vision. He also developed a right periorbital mass that was smaller in size. Computed tomography (CT) of the orbits showed bilateral lacrimal gland enlargement. Biopsy findings of the left periorbital mass showed a lymphoplasmacytic infiltrate with intervening areas of fibrosis, focal areas of necrobiosis, and Touton giant cells. Flow cytometry of this tissue showed a polyclonal B cell population. Two months prior to presentation, he developed shortness of breath and fatigue, and a chest radiograph demonstrated bilateral pleural effusions. The pleural fluid had a pH of 7.29. There were 164,000 red blood cells and 1,952 nucleated cells with a differential of 29% neutrophils, 52% lymphocytes, 7% monocytes, and 12% eosinophils. The glucose was less than 20 mg/dl. The protein was 5.7 gm/dl, and lactate dehydrogenase was 2,474 units/liter. Cultures and cytology findings were negative. Around the same time, he developed an erythematous, maculopapular rash on the lower extremities that resolved spontaneously in a week. He presented 2 months later to the rheumatology clinic for further evaluation. Past medical history and medicationsHe had a history of ulcerative colitis diagnosed at age 27 years, which was in remission on mesalamine, hyperlipidemia (lipid profile at age 5...

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IgG4‐related systemic disease and lymphoplasmacytic aortitis

Cited by 169 publications

References 35 publications

Association between IgG4-related disease and progressively transformed germinal centers of lymph nodes

Association between IgG4-related disease and progressively transformed germinal centers of lymph nodes

IgG4‐related systemic disease accounts for a significant proportion of thoracic lymphoplasmacytic aortitis cases

A 55‐Year‐Old Man With Periorbital and Inguinal Masses, Pericarditis, and Pleuritis

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