IgG4-related Disease Involving Multiple Organs with Elevated Serum Interleukin-6 Levels

Ikeura, Tsukasa; Horitani, Shunsuke; Masuda, Masataka; Kasai, Takeshi; Yanagawa, Masato; Miyoshi, Hideaki; Uchida, Kazushige; Takaoka, Makoto; Miyasaka, Chika; Uemura, Yoshiko; Okazaki, Kazuichi

doi:10.2169/internalmedicine.55.6919

Cited by 9 publications

(4 citation statements)

References 26 publications

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“…Excessive expression of RANKL was also reported in lymphoma [12], adult T cell leukemia [13], and chronic lymphocytic leukemia [14]. In IgG4RD, there are a number of reports which described an elevation of serum IL-6 [15][16][17]. However, an abnormality of serum Ca concentration was not reported.…”

Section: Discussionmentioning

confidence: 98%

A case of fatal osteolytic hypercalcemia complicated with IgG4-related ophthalmic disease leading to renal failure

et al. 2018

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A 40-year-old male was hospitalized with renal impairment and severe hypercalcemia. His concentration of serum IgG4 was high, but serum whole PTH, 1-25(OH) vitamin D3 and PTHrP were not elevated. Computed tomography showed swelling of the bilateral lacrimal glands and systemic lymphadenopathy. The histological findings of lacrimal gland biopsy fulfilled the diagnostic criteria of IgG4-related ophthalmic disease (IgG4ROD). Bone scintigraphy showed increased ectopic uptake in the stomach, heart, lungs, and kidneys. He died on day 16 of admission, although the therapies for hypercalcemia were continued. Autopsy results showed an increase of osteoclasts in the bone marrow and metastatic calcification in multiple organs, and excluded from the differential diagnosis other disorders which present lymph-node swelling and hypercalcemia such as cancer, lymphoma, Castleman's disease, and sarcoidosis. He was given a diagnosis of IgG4ROD with osteolytic hypercalcemia.

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Section: Discussionmentioning

confidence: 98%

A case of fatal osteolytic hypercalcemia complicated with IgG4-related ophthalmic disease leading to renal failure

et al. 2018

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“…Clinically, a favorable treatment response to PSL therapy may indicate a greater possibility of IgG4-RD than that of MCD. [39] Only few studies have reported IgG4-RD with elevated serum IL-6 levels [39][40][41], and the role of IL-6 in patients with IgG4-RD remains unclear. As IgG4 levels have not been reported for all previous cases of TAFRO syndrome, further studies are needed to discuss the association between TAFRO syndrome and elevated IgG4 levels.…”

Section: Discussionmentioning

confidence: 99%

Severe refractory TAFRO syndrome requiring continuous renal replacement therapy complicated with Trichosporon asahii infection in the lungs and myocardial infarction: an autopsy case report and literature review

et al. 2018

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Background: TAFRO (thrombocytopenia, anasarca, fever, reticulin myelofibrosis/renal failure, and organomegaly) syndrome is a systemic inflammatory disorder and unique clinicopathological variant of idiopathic multicentric Castleman disease that was proposed in Japan. Prompt diagnosis is critical because TAFRO syndrome is a progressive and life threating disease. Some cases are refractory to immunosuppressive treatments. Renal impairment is frequently observed in patients with TAFRO syndrome, and some severe cases require hemodialysis. Histological evaluation is important to understand the pathophysiology of TAFRO syndrome. However, systemic histopathological evaluation through autopsy in TAFRO syndrome has been rarely reported previously. Case presentation: A 46-year-old Japanese man with chief complaints of fever and abdominal distension was diagnosed with TAFRO syndrome through imaging studies, laboratory findings, and pathological findings on cervical lymph node and bone marrow biopsies. Interleukin (IL)-6 and vascular endothelial growth factor (VEGF) levels were remarkably elevated in both blood and ascites. Methylprednisolone (mPSL) pulse therapy was initiated on day 10, followed by combination therapy with PSL and cyclosporine A. However, the amount of ascites did not respond to the treatment. The patient became anuric, and continuous renal replacement therapy was initiated from day 50. However, the patient suddenly experienced cardiac arrest associated with myocardial infarction (MI) on the same day. Although the emergent percutaneous coronary intervention was successfully performed, the patient died on day 52, despite intensive care. Autopsy was performed to ascertain the cause of MI and to identify the histopathological characteristics of TAFRO syndrome.(Continued on next page)

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“…Characteristics of 10 patients with elevated serum IL-6 are shown in Table 3. Fig 1 shows CT images from a 63-year-old woman who complained of recurrent fever and exhibited elevated serum IL-6 (34 pg/mL) and was diagnosed with IgG4-RD [8]. Laboratory data at onset revealed anemia (hemoglobin, 7.8 g/dL), hypoalbuminemia (albumin, 1.5 g/dL), and elevated serum levels of IgG4 (1080 mg/dL) and CRP (5.05 mg/dL).…”

Section: Patient Characteristicsmentioning

confidence: 99%

“…IL-6, a principal mediator in inflammatory conditions, is reportedly a marker that is indicative of severity, progression, and outcome in several diseases [5]- [6]. Although MCD and IgG4-RD are thought to be distinct clinical entities, some patients with IgG4-RD exhibit elevated serum IL-6 [7]- [8], and in such patients it can be difficult to distinguish their condition from MCD.…”

Section: Introductionmentioning

confidence: 99%

Clinical implications of elevated serum interleukin-6 in IgG4-related disease

et al. 2020

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IntroductionSome patients with IgG4-related disease (IgG4-RD) exhibit elevated serum interleukin (IL)-6 with excessive inflammatory reactions or with repeating relapse. To date few reports pertaining to clinical implications of elevated serum IL-6 in IgG4-RD patients have been published. The aims of the current retrospective study were to investigate the clinical implications of elevated serum IL-6 in IgG4-RD patients, and to examine whether IL-6 can predict the activity and/or relapse of the disease. Materials and methodsWe examined the clinical picture at the onset of 43 patients who were diagnosed with IgG4-RD in our hospital and were able to measure serum IL-6 before steroid treatment. ResultsThe median level of serum IL-6 was 2.2 pg/mL. There was a significant correlation between IL-6 and C-reactive protein (CRP) level (r = 0.397, p = 0.008), hemoglobin level (r = -0.390, p = 0.010) and albumin level (r = -0.556, p < 0.001). When 43 patients were divided into two groups by using a cut-off IL-6 of 4 pg/mL, the high IL-6 group showed higher age, lower albumin, higher CRP and higher aspartate aminotransferase (AST) (age p = 0.014, albumin p = 0.006, CRP p <0.001, AST p = 0.009). Hepatic swelling and splenomegaly were significantly more prevalent in the high IL-6 group than it was in the low IL-6 group (liver p < 0.001, spleen p = 0.020). Biliary tract involvement tended to admit more in the high IL-6 group (p = 0.060).

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IgG4-related Disease Involving Multiple Organs with Elevated Serum Interleukin-6 Levels

Cited by 9 publications

References 26 publications

A case of fatal osteolytic hypercalcemia complicated with IgG4-related ophthalmic disease leading to renal failure

A case of fatal osteolytic hypercalcemia complicated with IgG4-related ophthalmic disease leading to renal failure

Severe refractory TAFRO syndrome requiring continuous renal replacement therapy complicated with Trichosporon asahii infection in the lungs and myocardial infarction: an autopsy case report and literature review

Clinical implications of elevated serum interleukin-6 in IgG4-related disease

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