• Title/Summary/Keyword: IgG4-Related Disease

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Rapidly resolved IgG4-related retroperitoneal fibrosis after steroid pulse therapy

  • Jeung, Soomin;Kim, Hyosang;Seo, Yuri;Yoon, Hee-Young;Lee, Nah Kyum;Park, Shinhee;Seo, Bomi;Park, Su-Yeon;Park, Su-Kil
    • Journal of Yeungnam Medical Science
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    • v.33 no.1
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    • pp.40-43
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    • 2016
  • Retroperitoneal fibrosis (RF) is a disorder characterized by the presence of a retroperitoneal mass and concurrent systemic inflammation. Some cases of RF are recognized as belonging to the spectrum of immunoglobulin G4-related disease (IgG4-RD). Glucocorticoids are highly effective for treatment of retroperitoneal fibrosis, although the optimal dose and duration of therapy have not been established. An initial dose of prednisone (40-60 mg) daily is usually administered with a tapering scheme. We report on a 55-year-old man diagnosed with IgG4-related RF and successfully treated with a 3-day course of daily 250 mg (4 mg/kg) intravenous methylprednisolone, which resulted in the prompt resolution of urinary obstruction and systemic symptoms.

Idiopathic multicentric Castleman disease presenting progressive reticular honeycomb infiltration of lung and immunoglobulin G and immunoglobulin G4 dominant hypergammaglobulinemia: a case report

  • Kim, Hyun-Je;Hong, Young-Hoon
    • Journal of Yeungnam Medical Science
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    • v.39 no.2
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    • pp.153-160
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    • 2022
  • Multicentric Castleman disease (MCD) is an uncommon systemic lymphoproliferative disorder that may cause multiple organ damage. Castleman disease-associated diffuse parenchymal lung disease (DPLD) has not been well studied. A 32-year-old man was referred to our hospital for progressive generalized weakness, light-headedness, and dyspnea on exertion for more than one year. Laboratory evaluations showed profound anemia, an elevated erythrocyte sedimentation rate, and an increased C-reactive protein level with polyclonal hypergammaglobulinemia. Chest radiography, computed tomography (CT), and positron emission tomography-CT scan demonstrated diffuse lung infiltration with multiple cystic lesions and multiple lymphadenopathy. In addition to these clinical laboratory findings, bone marrow, lung, and lymph node biopsies confirmed the diagnosis of idiopathic MCD (iMCD). Siltuximab, an interleukin-6 inhibitor, and glucocorticoid therapy were initiated. The patient has been tolerating the treatment well and had no disease progression or any complications in 4 years. Herein, we report this case of human herpesvirus-8-negative iMCD-associated DPLD accompanied by multiple cystic lesions, multiple lymphadenopathy, and polyclonal hypergammaglobulinemia with elevated immunoglobulin G (IgG) and IgG4 levels. We recommend a close evaluation of MCD in cases of DPLD with hypergammaglobulinemia.

Effects of BHOSB on Various Immunological Factors Related to Pathogenesis of Atopic Dermatitis in DNCB Treated NC/Nga Mice (비허온습만(脾虛蘊濕万)이 아토피 피부염 유발 NC/Nga 생쥐의 혈액내 면역 관련 인자에 미치는 영향)

  • Park, Sung-Hee;Jin, Mi-Rim;Koo, Young-Sun;Kim, Dong-Hee
    • Journal of Physiology & Pathology in Korean Medicine
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    • v.21 no.4
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    • pp.849-855
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    • 2007
  • Atopic dermatitis is an allergic inflammatory skin disease caused by aberrant and overreactive immune responses including overactivation of $T_H2$ immune responses, high levels of IgE as well as proinflammatory cytokines and chemokines. We examined whether BHOSB, a traditional herbal medicine, has modulatory effects on various immunological factors related to pathogenesis of atopic dermatitis in ONCB treated NC/Nga mice. Oral administration of BHOSB at the concentration of 10.8 mg/mouse/day significantly inhibited the production of IgE compared with control, and the levels of IgG2a and IgG2b, but not IgG1, were also significantly reduced. Production of IL-6 and TNF-a was greatly decreased. The results from flowcytometry of peripheral blood mononuclear cells indicated that the percentages of C03+C069+ cells and C04+ were significantly decreased by BHOSB. Theses results suggested that BHOSB has suppressive effects on aberrant and overreactive immunological activities in ONCB-induced dermatitis mice of NC/Nga.

Clinical and Radiological Features of Diffuse Lacrimal Gland Enlargement: Comparisons among Various Etiologies in 91 Biopsy-Confirmed Patients

  • Sae Rom Chung;Gye Jung Kim;Young Jun Choi;Kyung-Ja Cho;Chong Hyun Suh;Soo Chin Kim;Jung Hwan Baek;Jeong Hyun Lee;Min Kyu Yang;Ho-Seok Sa
    • Korean Journal of Radiology
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    • v.23 no.10
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    • pp.976-985
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    • 2022
  • Objective: To compare the clinical and radiological features of various etiologies of chronic diffuse lacrimal gland enlargement. Materials and Methods: We retrospectively reviewed 91 consecutive patients who underwent surgical biopsy for chronic diffuse lacrimal gland enlargement and were diagnosed with non-specific dacryoadenitis (DA) (n = 42), immunoglobulin G4-related dacryoadenitis (IgG4-RD) (n = 33), and lymphoma (n = 16). Data on patient demographics, clinical presentation, and CT imaging findings (n = 73) and MRI (n = 43) were collected. The following radiologic features of lacrimal gland enlargement were evaluated: size, unilaterality, wedge sign, angle with the orbital wall, heterogeneity, signal intensity, degree of enhancement, patterns of dynamic contrast-enhanced, and apparent diffusion coefficient value. Radiological features outside the lacrimal glands, such as extra-lacrimal orbital involvement and extra-orbital head and neck involvement, were also evaluated. The clinical and radiological findings were compared among the three diseases. Results: Compared to the DA and IgG4-RD groups, the lymphoma group was significantly older (mean 59.9 vs. 46.0 and 49.4 years, respectively; p = 0.001) and had a higher frequency of unilateral involvement (62.5% vs. 31.0% and 15.2%, respectively; p = 0.004). Compared to the IgG4-RD and lymphoma groups, the DA group had significantly smaller lacrimal glands (2.3 vs. 2.8 and 3.3 cm, respectively; p < 0.001) and a lower proportion of cases with a wedge sign (54.8% vs. 84.8% and 87.5%, respectively; p = 0.005). The IgG4-RD group showed more frequent involvement of the extra-orbital head and neck structures, including the infraorbital nerve (36.4%), paranasal sinus (72.7%), and salivary gland (58.6%) compared to the DA and lymphoma groups (4.8%-28.6%) (all p < 0.005). Conclusion: Patient age, unilaterality, lacrimal gland size, wedge sign, and extra-orbital head and neck involvement differed significantly different between lymphoma, DA, and IgG4-RD. Our results will be useful for the differential diagnosis and proper management of chronic lacrimal gland enlargement.

Interstitial Lung Disease (간질성 폐질환)

  • Chung, Man-Pyo
    • Tuberculosis and Respiratory Diseases
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    • v.71 no.3
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    • pp.163-171
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    • 2011
  • Recently published articles on interstitial lung disease (ILD) have focused on the accurate diagnosis of idiopathic pulmonary fibrosis (IPF), serum biomarkers, acute exacerbation of IPF, the prognostic factors of ILD and the trial of new treatment. In particular, reports on the serum biomarkers such as CC-chemokine ligand 18, surfactant protein, circulating fibrocytes, and acute exacerbation of IPF are sufficient to be mentioned here. Pirfenidone therapy is the most important trial for the treatment of IPF. Other newer treatment trials such as interferon-gamma, sildenafil and imatinib have been reported to be unsuccessful. On the other hand, the sirolimus trial for lymphangioleiomyomatosis is promising. Combined pulmonary fibrosis and emphysema and IgG4-related disease are established to be the new disease entities of ILD.

Experimental Study on Anti-allergic and Anti-inflammatory Effects of Soeumin-Googwihyangso-san Methanol Extract in Vitro (소음인(少陰人) 궁귀향소산(芎歸香蘇散)의 항(抗) 알레르기 및 항(抗) 염증에 미치는 실험적 연구)

  • Nam, Sang-Choon;Kang, Hee;Shim, Bum-Sang;Kim, Sung-Hoon;Choi, Seung-Hoon;Ahn, Kyoo-Seok
    • Journal of Physiology & Pathology in Korean Medicine
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    • v.23 no.1
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    • pp.41-49
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    • 2009
  • Soeumin-Googwihyangso-san(SGGHSS) has been used for the prevention or treatment of Soeumin-allergic rhinitis. This study was performed to demonstrate anti-allergic and anti-inflamatory effects of SGGHSS methanol extract in HMC cell lines and activated mouse B cells and CD4+ T cells. SGGHSS inhibited the production of TNF-$\alpha$ in PMA plus A23187 activated HMC-1 cells but not that of IL-6, as measured by ELISA. SGGHSS inhibited the expression of CD23 and surface IgE in B cells as determined by flowcytometry. It also inhibited secretion of IFN-$\gamma$ and IgG1, the Th1 related IgG type, but increased that of IL-4 in anti-CD40 and IL-4 treated B cells as measured by ELISA. As for Th cell differentiation, SGGHSS did not much affect IL-4 or IFN-$\gamma$. Taken together, our data showed that SGGHSS exerted an anti-inflammtory effect by inhibiting TNF-$\alpha$ in mast cells and has anti-allergic activity not via inhibition of CD4+ T cell, but via inhibition of B cells. These results suggest some evidence that SGGHSS can be applied to allergic disease.

Comparison of serum amyloid A protein and C-reactive protein levels as inflammatory markers in periodontitis

  • Ardila, Carlos Martin;Guzman, Isabel Cristina
    • Journal of Periodontal and Implant Science
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    • v.45 no.1
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    • pp.14-22
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    • 2015
  • Purpose: The purpose of this study was to compare serum amyloid A (SAA) protein levels with high-sensitive C-reactive protein (hs-CRP) levels as markers of systemic inflammation in patients with chronic periodontitis. The association of serum titers of antibodies to periodontal microbiota and SAA/hs-CRP levels in periodontitis patients was also studied. Methods: A total of 110 individuals were included in this study. Patients were assessed for levels of hs-CRP and SAA. Nonfasting blood samples were collected from participants at the time of clinical examination. The diagnosis of adipose tissue disorders was made according to previously defined criteria. To determine SAA levels, a sandwich enzyme-linked immunosorbent assay was utilized. Paper points were transferred to a sterile tube to obtain a pool of samples for polymerase chain reaction processing and the identification of Porphyromonas gingivalis, Aggregatibacter actinomycetemcomitans, and Tannerella forsythia. The serum level of IgG1 and IgG2 antibodies to P. gingivalis, A. actinomycetemcomitans, and T. forsythia was also determined. Results: SAA and hs-CRP levels were higher in periodontitis patients than in controls (P<0.05). In bivariate analysis, high levels of hs-CRP (>3 mg/L) and SAA (>10 mg/L) were significantly associated with chronic periodontitis (P=0.004). The Spearman correlation analysis between acute-phase proteins showed that SAA positively correlated with hs-CRP (r=0.218, P=0.02). In the adjusted model, chronic periodontitis was associated with high levels of SAA (odds ratio [OR], 5.5; 95% confidence interval [CI], 1.6-18.2; P=0.005) and elevated hs-CRP levels (OR, 6.1, 95% CI, 1.6-23.6; P=0.008). Increased levels of serum IgG2 antibodies to P. gingivalis were associated with high levels of SAA (OR, 3.6; 95% CI, 1.4-8.5; P=0.005) and high concentrations of hs-CRP (OR, 4.3; 95% CI, 1.9-9.8; P<0.001). Conclusions: SAA and hs-CRP concentrations in patients with chronic periodontitis are comparably elevated. High serum titers of antibodies to P. gingivalis and the presence of periodontal disease are independently related to high SAA and hs-CRP levels.

Association between periodontal bacteria and degenerative aortic stenosis: a pilot study

  • Kataoka, Akihisa;Katagiri, Sayaka;Kawashima, Hideyuki;Nagura, Fukuko;Nara, Yugo;Hioki, Hirofumi;Nakashima, Makoto;Sasaki, Naoki;Hatasa, Masahiro;Maekawa, Shogo;Ohsugi, Yujin;Shiba, Takahiko;Watanabe, Yusuke;Shimokawa, Tomoki;Iwata, Takanori;Kozuma, Ken
    • Journal of Periodontal and Implant Science
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    • v.51 no.4
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    • pp.226-238
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    • 2021
  • Purpose: Although several reports have described the relationship between periodontal disease and cardiovascular disease, information about the association between periodontal disease and the progression of degenerative aortic stenosis (AS) is lacking. Therefore, we performed a retrospective, single-center, pilot study to provide insight into this potential association. Methods: Data from 45 consecutive patients (19 men; median age, 83 years) with mild or moderate degenerative aortic stenosis were analyzed for a mean observation period of 3.3±1.9 years. The total amount of Aggregatibacter actinomycetemcomitans and Porphyromonas gingivalis and titers of serum immunoglobulin G (IgG) against periodontal bacteria and high-sensitivity C-reactive protein (hs-CRP) were evaluated. Aortic valve area (AVA), maximal velocity (Vmax), mean pressure gradient (mean PG), and the Doppler velocity index (DVI) were evaluated. The change in each parameter per year ([ParameterLATEST-ParameterBASELINE]/Follow-up Years) was calculated from the retrospective follow-up echocardiographic data (baseline vs. the most recently collected data [latest]). Results: No correlation was found between the concentration of periodontopathic bacteria in the saliva and AS status/progression. The anti-P. gingivalis antibody titer in the serum showed a significant positive correlation with AVA and DVI. Additionally, there was a negative correlation between the anti-P. gingivalis IgG antibody titer and mean PG. The hs-CRP concentration showed positive correlations with Vmax and mean PG. Meanwhile, a negative correlation was observed between the anti-P. gingivalis IgG antibody titer and ΔAVA/year and Δmean PG/year. The hs-CRP concentration showed positive correlations with Vmax and mean PG, and it was significantly higher in patients with rapid aortic stenosis progression (ΔAVA/year <-0.1) than in their counterparts. Conclusions: Our results suggest that periodontopathic bacteria such as A. actinomycetemcomitans and P. gingivalis are not directly related to the status/progression of degenerative AS. However, inflammation and a lower immune response may be associated with disease progression.

Multi-Detector CT Findings of Typical and Atypical Appendicitis: A Pictorial Essay (전형적 및 비전형적 충수염의 다중검출기컴퓨터단층촬영 소견: 임상화보)

  • Na Ri Yun;You Dong Won;Su Lim Lee
    • Journal of the Korean Society of Radiology
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    • v.84 no.5
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    • pp.1047-1065
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    • 2023
  • Multi-detector CT (MDCT) is a highly accurate diagnostic tool that is commonly used to evaluate appendicitis and its complications. The diagnosis of appendicitis based on MDCT findings can be difficult and challenging when the observed findings are inconsistent with the typical features. Atypical appendicitis includes a wide spectrum of features, such as variable positions of the appendix and cecum, complications, and unusual pathological findings of secondary appendicitis that mimic or induce appendicitis. Our pictorial essay describes the diverse spectrum of atypical appendicitis and appendicitis-like conditions in terms of location abnormalities, complications, and uncommon pathologies, including related tumors, reactive appendicitis, appendiceal diverticulitis, and IgG4-related disease. Through this essay, the readers can become more familiar with MDCT findings of atypical appendicitis.

Immunologic Alteration Demonstrated at the Economic Animal Husbandry Workers (면역독성학적 분석에 의한 축산업 종사자들의 건강 유해성 평가)

  • Kim, Hyoung-Ah;Lee, Kyung-Sook;Kim, Kyung-Ran;Kim, Kwang-Ho;Heo, Yong
    • Toxicological Research
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    • v.21 no.2
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    • pp.121-128
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    • 2005
  • Economic animal husbandry workers exposed to organic dust can be suffered from immunologic disorders. Our study was to determine immunological parameters related with occurrence of respiratory allergic diseases to animal husbandry workers in Korea for the first time. Peripheral blood were obtained from twenty-five pig barn workers, forty-nine chicken farming workers and fifty-one non-agricultural control workers. Significantly upregulated plasma IgE level was observed with pig-barn workers than that of chicken farming workers or healthy community control subjects. Furthermore, level of histamine, a hallmark of allergy induction, was upregulated in the pig and chicken farming workers in comparison with that of the control subjects. Downregulation of $IFN_\gamma$ and $TNF_{\alpha}$ production from T cells was apparent in the animal husbandry workers compared with the control subjects. Meanwhile, T cells collected from the pig barn workers demonstrated significantly higher production of IL-4 and IL-10 than the other groups. There were also alterations in IgG subclass distribution. In conclusion, immunological modulation probably leading to occupational allergic diseases can be occurred in the economic animal husbandry workers and the pig barn workers could be the most risky group to the work-related allergic disease.