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1. The 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-related disease

Author

Wallace, Zachary S, Naden, Ray P, Chari, Suresh, Choi, Hyon K, Della-Torre, Emanuel, Dicaire, Jean-Francois, Hart, Phillip A, Inoue, Dai, Kawano, Mitsuhiro, Khosroshahi, Arezou, Lanzillotta, Marco, Okazaki, Kazuichi, Perugino, Cory A, Sharma, Amita, Saeki, Takako, Schleinitz, Nicolas, Takahashi, Naoki, Umehara, Hisanori, Zen, Yoh, Stone, Collaborators: Akamizu T, John H., Akiyama, M, Barra, L, Bateman, A, Blockmans, D, Brito-Zeron, P, Campochiaro, C, Carruthers, M, Chiba, T, Cornell, L, Culver, E, Darabian, S, Deshpande, V, Dong, L, Ebbo, M, Fernández-Codina, A, Ferry, Ja, Fragkoulis, G, Frost, F, Frulloni, Luca, Hernandez-Molina, G, Ji, H, Keat, K, Kamisawa, T, Kawa, S, Kobayashi, H, Kodama, Y, Kubo, S, Kubota, K, Leng, H, Lerch, Mm, Liu, Y, Liu, Z, Löhr, M, Martin-Nares, E, Martinez-Valle, F, Marvisi, C, Masaki, Y, Matsui, S, Mizushima, I, Nakamura, S, Nordeide, J, Notohara, K, Paira, S, Popovic, J, Ramos-Casals, M, Rosenbaum, J, Ryu, J, Sato, Y, Sekiguchi, H, Sokol, Ev, Stone, Jr, Sun, W, Takahashi, H, Takahira, M, Tanaka, Y, Vaglio, A, Villamil, A, Wada, Y, Webster, G, Yamada, K, Yamamoto, M, Yi, J, Yi, Y, Zamboni, G, Zhang, W., Wallace, Z, Naden, Rp, Chari, S, Choi, Hk, DELLA TORRE, E, Dicaire, Jf, Hart, Pa, Inoue, D, Kawano, M, Khosroshahi, A, Lanzillotta, M, Okazaki, K, Perugino, Ca, Sharma, A, Saeki, T, Schleinitz, N, Takahashi, N, Umehara, H, Zen, Y, Stone, Jh, and Members of the ACR/EULAR IgG4-RD Classification Criteria Working, Group.

Subjects
rheumatoid arthritis, Adult, Male, 0301 basic medicine, medicine.medical_specialty, Immunology, Sensitivity and Specificity, General Biochemistry, Genetics and Molecular Biology, Diagnosis, Differential, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Internal medicine, parasitic diseases, Epidemiology, Humans, Immunology and Allergy, Medicine, Pathological, Sjøgren's syndrome, Aged, 030203 arthritis & rheumatology, business.industry, Middle Aged, inflammation, medicine.disease, Test (assessment), 030104 developmental biology, Rheumatoid arthritis, Radiological weapon, Female, Immunoglobulin G4-Related Disease, business, Rheumatism, Decision analysis
Abstract

IgG4-related disease (IgG4-RD) can cause fibroinflammatory lesions in nearly any organ. Correlation among clinical, serological, radiological and pathological data is required for diagnosis. This work was undertaken to develop and validate an international set of classification criteria for IgG4-RD. An international multispecialty group of 86 physicians was assembled by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). Investigators used consensus exercises; existing literature; derivation and validation cohorts of 1879 subjects (1086 cases, 793 mimickers); and multicriterion decision analysis to identify, weight and test potential classification criteria. Two independent validation cohorts were included. A three-step classification process was developed. First, it must be demonstrated that a potential IgG4-RD case has involvement of at least one of 11 possible organs in a manner consistent with IgG4-RD. Second, exclusion criteria consisting of a total of 32 clinical, serological, radiological and pathological items must be applied; the presence of any of these criteria eliminates the patient from IgG4-RD classification. Third, eight weighted inclusion criteria domains, addressing clinical findings, serological results, radiological assessments and pathological interpretations, are applied. In the first validation cohort, a threshold of 20 points had a specificity of 99.2% (95% CI 97.2% to 99.8%) and a sensitivity of 85.5% (95% CI 81.9% to 88.5%). In the second, the specificity was 97.8% (95% CI 93.7% to 99.2%) and the sensitivity was 82.0% (95% CI 77.0% to 86.1%). The criteria were shown to have robust test characteristics over a wide range of thresholds. ACR/EULAR classification criteria for IgG4-RD have been developed and validated in a large cohort of patients. These criteria demonstrate excellent test performance and should contribute substantially to future clinical, epidemiological and basic science investigations.

Published
2019
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2. The 2019 American College of Rheumatology/European League Against Rheumatism Classification Criteria for IgG4-Related Disease

Author

Wallace, Zachary S, Naden, Ray P, Chari, Suresh, Choi, Hyon, Della-Torre, Emanuel, Dicaire, Jean-Francois, Hart, Phil A, Inoue, Dai, Kawano, Mitsuhiro, Khosroshahi, Arezou, Kubota, Kensuke, Lanzillotta, Marco, Okazaki, Kazuichi, Perugino, Cory A, Sharma, Amita, Saeki, Takako, Sekiguchi, Hiroshi, Schleinitz, Nicolas, Stone, James R, Takahashi, Naoki, Umehara, Hisanori, Webster, George, Zen, Yoh, Stone, John H, Akamizu, T, Akiyama, M, Barra, L, Bateman, A, Blockmans, D, Brito-Zeron, P, Campochiaro, C, Carruthers, M, Chari, S, Chiba, T, Choi, H, Cornell, L, Culver, E, Darabian, S, Torre, Ed, Deshpande, V, Dong, L, Ebbo, M, Fernández-Codina, A, Ferry, Ja, Fragkoulis, G, Frost, F, Frulloni, L, Hart, Pa, Hernandez-Molina, G, Inoue, D, Ji, H, Keat, K, Kamisawa, T, Kawa, S, Kawano, M, Khosroshahi, A, Kobayashi, H, Kodama, Y, Kubo, S, Kubota, K, Lanzillotta, M, Leng, H, Lerch, M, Liu, Y, Liu, Z, Löhr, M, Martin-Nares, E, Martinez-Valle, F, Marvisi, C, Masaki, Y, Matsui, S, Mizushima, I, Naden, Rp, Nakamura, S, Nordeide, J, Notohara, K, Okazaki, K, Paira, S, Perugino, Ca, Popovic, J, Ramos-Casals, M, Rosenbaum, J, Ryu, J, Saeki, T, Sato, Y, Schleinitz, N, Sekiguchi, H, Sharma, A, Sokol, Ev, Stone, Jr, Stone, Jh, Sun, W, Takahashi, H, Takahashi, N, Takahira, M, Tanaka, Y, Umehara, H, Vaglio, A, Villamil, A, Wada, Y, Wallace, Zs, Webster, G, Yamada, K, Yamamoto, M, Yi, J, Yi, Y, Zamboni, G, Zen, Y, Zhang, W., Wallace, Z, Naden, Rp, Chari, S, Choi, H, DELLA TORRE, E, Dicaire, Jf, Hart, Pa, Inoue, D, Kawano, M, Khosroshahi, A, Kubota, K, Lanzillotta, M, Okazaki, K, Perugino, Ca, Sharma, A, Saeki, T, Sekiguchi, H, Schleinitz, N, Stone, Jr, Takahashi, N, Umehara, H, Webster, G, Zen, Y, Stone, Jh, and American College of Rheumatology/European League Against Rheumatism IgG4-Related Disease Classification Criteria Working, Group.

Subjects
Adult, Male, medicine.medical_specialty, Consensus, Aged, Diagnosis, Differential, Europe, Female, Humans, Immunoglobulin G4-Related Disease, Middle Aged, Reproducibility of Results, Rheumatology, Societies, Medical, United States, Immunology, MEDLINE, Disease, Internal medicine, Medical, parasitic diseases, Diagnosis, Immunology and Allergy, Medicine, business.industry, medicine.disease, Confidence interval, Test (assessment), Differential, IgG4-related disease, business, Societies, Rheumatism, Decision analysis
Abstract

Objective IgG4-related disease (IgG4-RD) can cause fibroinflammatory lesions in nearly any organ. Correlation among clinical, serologic, radiologic, and pathologic data is required for diagnosis. This work was undertaken to develop and validate an international set of classification criteria for IgG4-RD. Methods An international multispecialty group of 86 physicians was assembled by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). Investigators used consensus exercises, existing literature, derivation and validation cohorts of 1,879 subjects (1,086 cases, 793 mimickers), and multicriterion decision analysis to identify, weight, and test potential classification criteria. Two independent validation cohorts were included. Results A 3-step classification process was developed. First, it must be demonstrated that a potential IgG4-RD case has involvement of at least 1 of 11 possible organs in a manner consistent with IgG4-RD. Second, exclusion criteria consisting of a total of 32 clinical, serologic, radiologic, and pathologic items must be applied; the presence of any of these criteria eliminates the patient from IgG4-RD classification. Third, 8 weighted inclusion criteria domains, addressing clinical findings, serologic results, radiology assessments, and pathology interpretations, are applied. In the first validation cohort, a threshold of 20 points had a specificity of 99.2% (95% confidence interval [95% CI] 97.2-99.8%) and a sensitivity of 85.5% (95% CI 81.9-88.5%). In the second, the specificity was 97.8% (95% CI 93.7-99.2%) and the sensitivity was 82.0% (95% CI 77.0-86.1%). The criteria were shown to have robust test characteristics over a wide range of thresholds. Conclusion ACR/EULAR classification criteria for IgG4-RD have been developed and validated in a large cohort of patients. These criteria demonstrate excellent test performance and should contribute substantially to future clinical, epidemiologic, and basic science investigations.

Published
2018

3. The role of anticyclic citrullinated peptide antibodies in the differential diagnosis of elderly-onset rheumatoid arthritis and polymyalgia rheumatica.

Author

Ceccato, F., Roverano, S., Barrionuevo, A., Rillo, O., and Paira, S.

Subjects
RHEUMATOID arthritis, POLYMYALGIA rheumatica, DIFFERENTIAL diagnosis, IMMUNOGLOBULINS, RHEUMATISM
Abstract

There are clinical difficulties to differentiate elderly-onset rheumatoid arthritis (EORA) patients from those with polymyalgia rheumatica (PMR), especially when dealing with EORA-like PMR-onset, seronegative EORA, and PMR with peripheral synovitis, which constitute the subgroups presenting the greatest difficulties. Serum samples were obtained from two groups of patients, one with EORA diagnosis and another with a PMR diagnosis. Anticyclic citrullinated peptide (anti-CCP) antibodies (enzyme-linked immunosorbent assay method) and rheumatoid factor (RF; latex technique) were determined. Of the 16 EORA patients, 9 presented anti-CCP antibodies, 4 of whom tested positive for RF. Of the 12 EORA patients who remained negative to RF, 5 were positive for anti-CCP antibodies. Eight of the EORA patients started with polymyalgic symptoms. Three of these patients showed positive titles of anti-CCP antibodies with negative RF. All PMR patients presented negative anti-CCP antibodies, except one with weak positive titles, and all were negative for RF. Of 15 patients with PMR, 7 presented oligoarticular synovitis at the onset. After a mean follow-up of 3 months, two patients developed RA. When evaluating them for RF and anti-CCP antibodies, one tested negative, while the other was positive for both antibodies. We observed a tendency to higher values of anti-CCP antibodies in patients with extraarticular manifestations, radiological damage, and disease-modifying antirheumatic drugs. When compared to the PMR group, EORA patients presented positive anticitrulline antibodies at the beginning of the disease in a statistically significant amount. One third of the seronegative EORA patients presented positive anti-CCP antibodies at the onset. [ABSTRACT FROM AUTHOR]

Published
2006
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4. Distal Extremity Swelling with Pitting Oedema in Rheumatoid Arthritis.

Author

Paira, S., Caliani, L., and Luraquiz, N.

Subjects
RHEUMATOID arthritis, EDEMA, ARTHRITIS, RHEUMATISM, AUTOIMMUNE diseases, RHEUMATOLOGY
Abstract

: Distal extremity swelling with pitting oedema due to altered lymphatic drainage has been reported in some patients with rheumatoid arthritis (RA). The resistant-to-therapy oedema usually affected the upper limbs in an asymmetrical pattern. Until now, extensor tenosynovial involvement has not been described in RA patients suffering from distal extremity swelling with pitting oedema. Three patients are described: two of them had predominant extensor tenosynovial involvement in their hands, with impaired lymphatic drainage demonstrated by (MRI) and lymphoscintigraphy, respectively. In both cases the oedema was chronic and not responsive to treatment. One patient had extensor tenosynovial involvement without impaired lymphatic drainage. In this case, the oedema remitted completely after a few days of corticosteroid therapy. None of them showed differences in serum levels of vascular endothelial growth factor (VEGF), whether they were RA patients with no pitting oedema or healthy volunteers. [ABSTRACT FROM AUTHOR]

Published
2001
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5. Unusual manifestation of histoplasmosis in connective tissue diseases.

Author

Ceccato, F., Gongora, V., Zunino, A., Roverano, S., and Paira, S.

Subjects
HISTOPLASMOSIS, MUSCULOSKELETAL system, RHEUMATISM, AUTOIMMUNE diseases, VASCULAR diseases, RHEUMATOID arthritis, MYOSITIS
Abstract

This report describes the coexistence of three patients with rheumatic diseases (systemic lupus erythematosus, rheumatoid arthritis, and dermatomyositis) and infections because of Histoplasma capsulatum. Connective tissue diseases and histoplasmosis share several clinical findings. Therefore, histoplasmosis could be misdiagnosed as connective tissue disease or a flare of these diseases. Such cases highlight the importance of awareness of histoplasmosis in immunocompromised patients, particularly in those originating from endemic areas. [ABSTRACT FROM AUTHOR]

Published
2007
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