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

Stone, John H.; Khosroshahi, Arezou; Stone, James R.

doi:10.1002/acr.20095

Cited by 148 publications

(126 citation statements)

References 34 publications

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“…When evaluating aortic specimens, we propose that a cell ratio of 450% be considered as a minimum criterion, because some cases of atherosclerosis and giant cell or infectious aortitis can show IgG4 þ /IgG þ ratios close to 40%. 51,52 The majority of cases that fulfill these criteria will show clinical and serological findings that are typical for IgG4-related disease. In the minority of cases that lack these criteria, histopathology would be the defining feature.…”

Section: Histologically Highly Suggestive Of Igg4-related Diseasementioning

confidence: 99%

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Consensus statement on the pathology of IgG4-related disease

et al. 2012

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IgG4-related disease is a newly recognized fibro-inflammatory condition characterized by several features: a tendency to form tumefactive lesions in multiple sites; a characteristic histopathological appearance; andoften but not always-elevated serum IgG4 concentrations. An international symposium on IgG4-related The histopathology of IgG4-related disease was a specific focus of the international symposium. The primary purpose of this statement is to provide practicing pathologists with a set of guidelines for the diagnosis of IgG4-related disease. The diagnosis of IgG4-related disease rests on the combined presence of the characteristic histopathological appearance and increased numbers of IgG4 þ plasma cells. The critical histopathological features are a dense lymphoplasmacytic infiltrate, a storiform pattern of fibrosis, and obliterative phlebitis. We propose a terminology scheme for the diagnosis of IgG4-related disease that is based primarily on the morphological appearance on biopsy. Tissue IgG4 counts and IgG4:IgG ratios are secondary in importance. The guidelines proposed in this statement do not supplant careful clinicopathological correlation and sound clinical judgment. As the spectrum of this disease continues to expand, we advocate the use of strict criteria for accepting newly proposed entities or sites as components of the IgG4-related disease spectrum.

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Section: Histologically Highly Suggestive Of Igg4-related Diseasementioning

confidence: 99%

“…6,8,10,12,[15][16][17]27,28,30,32,34,35,49,51,52,[55][56][57][58][59][60][61][62][63] Consensus statement on the pathology of IgG4-related disease V Deshpande et al which is designed to bind to the Fc portion of IgG4 molecules. 7,10,11,31,40,41 Polyclonal antibodies have been also used by some groups.…”

Section: Technical Issues-igg4 Immunostainmentioning

confidence: 99%

Consensus statement on the pathology of IgG4-related disease

et al. 2012

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“…[1][2][3][4][5][6] IgG4-related aortitis/periaortitis appears to occur in two forms. 6 In some cases, there is an outright aortitis with evidence of systemic IgG4-related disease including elevated serum levels of IgG4.…”

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confidence: 99%

An elevated IgG4 response in chronic infectious aortitis is associated with aortic atherosclerosis

2015

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Recently, it was shown that infectious bacterial aortitis can stimulate an elevated IgG4 + plasma cell response in the vessel wall, which could mimic IgG4 aortitis/periaortitis. However, the factors that are associated with an elevated IgG4 + plasma cell response in infectious aortitis are unclear. To ascertain these factors, 17 cases of infectious aortitis and 6 cases of non-infectious severe abdominal aortic atherosclerosis were assessed for the magnitude of IgG4 + plasma cell response. The degree of IgG4 + plasma cell infiltration was determined by immunohistochemistry. Infectious cases were subcharacterized as chronic (43 weeks duration) or acute ( o3 weeks duration) based on the duration of symptoms, and as involving either the ascending aorta or the distal aorta, ie, the descending thoracic and/or abdominal aorta. There was a 5-16-fold greater degree of IgG4 + plasma cell infiltration in the chronic distal infectious aortitis group compared with the other three infectious aortitis groups (P ≤ 0.0007), and compared with non-infectious severe abdominal aortic atherosclerosis (Po 0.0008). This resulted in a greater IgG4/IgG ratio in the chronic distal infectious aortitis group compared with the acute ascending and acute distal infectious aortitis groups (Po 0.03). The degree of IgG4 + plasma cell infiltration in chronic distal infectious aortitis overlaps with that seen in the aortitis and periaortitis of IgG4-related disease. In the chronic infectious aortitis cases, the degree of IgG4 + plasma cell infiltration was more intense in patients with moderate to severe aortic atherosclerosis compared with those patients with less aortic atherosclerosis (P = 0.007). These findings indicate that an elevated IgG4 + plasma cell response occurs in the descending thoracic and abdominal aorta in the setting of chronic bacterial infectious aortitis and pre-existing atherosclerosis. This inflammatory response to chronic infection in atherosclerosis-laden aortas may have implications for the development of IgG4-rich inflammatory atherosclerotic aortic aneurysms.

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“…Many different terms have been used to describe conditions that have become more recently recognized as members of a spectrum of clinical involvement in IgG4-related systemic disease (31,(35)(36)(37)(38). Full recognition of the disease is challenging, as involvement of different organs affected is often metachronous.…”

Section: Discussionmentioning

confidence: 99%

“…Stone et al found that IgG4-positive lymphoplasmacytic infiltrate is present in cases of aortitis (37). During a 5-year period at Massachusetts General Hospital, 5.2% of thoracic aortitis cases were noninfectious.…”

Section: Discussionmentioning

confidence: 99%

A 45‐year‐old man with flank pain and inability to ejaculate

Muskardin

Gertner

2012

Arthritis Care & Research

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A 45-year-old man with left-sided flank pain and difficulty ejaculating. History of the Present IllnessThe patient developed left-sided flank pain in April 2010. He was evaluated in the emergency department when the pain had persisted for 3 weeks. He reported worsening of the pain in the evening and difficulty with ejaculation since the pain began: "I get a knot in my side," then "I can't ejaculate." He also reported associated low back pain and increased pain with bending to his left. His general physical examination was unremarkable. Urine analysis demonstrated 30 mg/dl protein, 10 mg/dl ketones, 8 mg/dl urobilinogen (reference value Ͻ2.0), 8 red blood cells/ high-power field (hpf), 2 white blood cells/hpf, and mucous. He was prescribed ibuprofen for abdominal pain and was advised to follow up with his primary care provider.The pain persisted. He saw his primary provider in July with worsening epigastric and left-sided abdominal and flank pain. By this time, he had decreased appetite, early satiety, and weight loss of 22 pounds. His primary provider ordered a computed tomographic (CT) scan of his abdomen and pelvis, which demonstrated a rind of soft tissue in the retroperitoneum surrounding the aorta and inferior vena cava and wall thickening of the sigmoid colon and rectum. A biopsy of the mass was done by interventional radiology at another hospital. Since his pain was unrelieved by acetaminophen/hydrocodone, he was admitted to our hospital for further evaluation and pain control. He did not know the results of the biopsy. Medical HistoryHe had been relatively healthy prior to the onset of illness in April. Atheromatous calcification of the aorta with mild ectasia inferiorly measuring up to 2.3 cm in diameter was noted incidentally on a CT scan obtained to evaluate for ureteral calculi in September 2008. He had a remote history of gonorrhea and chlamydia infections, which were treated. He had been seen in the emergency department for right testicular pain following trauma at age 35 years and left testicular pain without clear etiology at ages 40 and 43 years. The first episode of left testicular pain was attributed to epididymitis. The second was attributed to suspected passage of a ureteral calculus. Social and Family HistoryThe patient's family history was negative for rheumatic disorders. Several family members, including his mother, had diabetes mellitus and hypertension. His sister had thyroid disease. He was unemployed, but previously worked as a baker. He smoked one-quarter of a pack of cigarettes per day for several years, and previously smoked marijuana. He consumed, on average, 1 alcoholic beverage per week and denied intravenous drug use. He was sexually active and did not consistently use barrier protection. Review of SystemsThe patient reported persistent inability to ejaculate, without decrease in arousal or orgasm and without associated pain. He also reported low back pain that preceded his current illness by years. He had constipation for 2 weeks.

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

Cited by 148 publications

References 34 publications

Consensus statement on the pathology of IgG4-related disease

Consensus statement on the pathology of IgG4-related disease

An elevated IgG4 response in chronic infectious aortitis is associated with aortic atherosclerosis

A 45‐year‐old man with flank pain and inability to ejaculate

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