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Article ; Online: Impact of age and cardiovascular risk factors on the incidence of adverse events in patients with rheumatoid arthritis treated with Janus Kinase inhibitors: data from a real-life multicentric cohort.

Gentileschi, Stefano / Gaggiano, Carla / Damiani, Arianna / Coccia, Carmela / Bernardini, Pamela / Cazzato, Massimiliano / D'Alessandro, Francesco / Vallifuoco, Giulia / Terribili, Riccardo / Bardelli, Marco / Baldi, Caterina / Cantarini, Luca / Mosca, Marta / Frediani, Bruno / Guiducci, Serena

Clinical and experimental medicine

2024 Volume 24, Issue 1, Page(s) 62

Abstract: Inhibiting Janus Kinases (JAK) is a crucial therapeutic strategy in rheumatoid arthritis (RA). However, the use of JAK inhibitors has recently raised serious safety concerns. The study aims to evaluate the safety profile of JAKi in patients with RA and ... ...

Abstract	Inhibiting Janus Kinases (JAK) is a crucial therapeutic strategy in rheumatoid arthritis (RA). However, the use of JAK inhibitors has recently raised serious safety concerns. The study aims to evaluate the safety profile of JAKi in patients with RA and identify potential risk factors (RFs) for adverse events (AEs). Data of RA patients treated with JAKi in three Italian centers from January 2017 to December 2022 were retrospectively analyzed. 182 subjects (F:117, 64.3%) underwent 193 treatment courses. 78.6% had at least one RF, including age ≥ 65 years, obesity, smoking habit, hypertension, dyslipidemia, hyperuricemia, diabetes, previous VTE or cancer, and severe mobility impairment. We identified 70 AEs (28/100 patients/year), among which 15 were serious (6/100 patients/year). A high disease activity was associated with AEs occurrence (p = 0.03 for CDAI at T0 and T6; p = 0.04 for SDAI at T0 and T6; p = 0.01 and p = 0.04 for DAS28ESR at T6 and T12, respectively). No significant differences in AEs occurrence were observed after stratification by JAKi molecules (p = 0.44), age groups (p = 0.08) nor presence of RFs (p > 0.05 for all of them). Neither the presence of any RFs, nor the cumulative number of RFs shown by the patient, nor age ≥ 65 did predict AEs occurrence. Although limited by the small sample size and the limited number of cardiovascular events, our data do not support the correlation between cardiovascular RFs-including age-and a higher incidence of AEs during JAKi therapy. The role of uncontrolled disease activity in AEs occurrence should by emphasized.
MeSH term(s)	Humans ; Aged ; Janus Kinase Inhibitors/adverse effects ; Cardiovascular Diseases/epidemiology ; Incidence ; Retrospective Studies ; Risk Factors ; Arthritis, Rheumatoid/drug therapy ; Heart Disease Risk Factors ; Antirheumatic Agents/adverse effects
Chemical Substances	Janus Kinase Inhibitors ; Antirheumatic Agents
Language	English
Publishing date	2024-03-30
Publishing country	Italy
Document type	Journal Article
ZDB-ID	2053018-3
ISSN	1591-9528 ; 1591-8890
ISSN (online)	1591-9528
ISSN	1591-8890
DOI	10.1007/s10238-024-01325-z
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Article: ReLiFiRa (Real Life Filgotinib in Rheumatoid Arthritis): Retrospective Study of Efficacy and Safety in Common Clinical Practice.

Benucci, Maurizio / Bardelli, Marco / Cazzato, Massimiliano / Laurino, Elenia / Bartoli, Francesca / Damiani, Arianna / Li Gobbi, Francesca / Panaccione, Anna / Di Cato, Luca / Niccoli, Laura / Frediani, Bruno / Mosca, Marta / Guiducci, Serena / Cantini, Fabrizio

Journal of personalized medicine

2023 Volume 13, Issue 9

Abstract: Background: Filgotinib (FIL) is a selective JAK1 inhibitor with an affinity 30-fold higher than JAK2, approved to treat moderate to severe active rheumatoid arthritis (RA), in adults with inadequate response or intolerance to one or more disease- ... ...

Abstract	Background: Filgotinib (FIL) is a selective JAK1 inhibitor with an affinity 30-fold higher than JAK2, approved to treat moderate to severe active rheumatoid arthritis (RA), in adults with inadequate response or intolerance to one or more disease-modifying antirheumatic drugs (DMARDs). Methods: We conducted a retrospective, multicentric study in order to evaluate efficacy and safety of FIL 200 mg daily therapy, after 3 and 6 months, in 120 patients affected by RA, managed in Tuscany and Umbria rheumatological centers. The following clinical records were analyzed: demographical data, smoking status, previous presence of comorbidities (Herpes zoster -HZ- infection, venous thromboembolism -VTE-, major adverse cardiovascular events -MACE-, cancer, diabetes, and hypertension), disease duration, presence of anti-citrullinated protein antibodies (ACPA), rheumatoid factor (RF), number of biological failures, and prior csDMARDs utilized. At baseline, and after 3 (T3) and 6 (T6) months of FIL therapy, we evaluated mean steroid dosage, csDMARDs intake, clinimetric indexes (DAS28, CDAI, HAQ, patient and doctor PGA, VAS), erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and body mass index (BMI). Results: At baseline, the mean disease duration was 9.4 ± 7.5 years; the prevalence of previous HZ infection, VTE, MACE, and cancer was respectively 4.12%, 0%, 7.21%, and 0.83%, respectively. In total, 76.3% of patients failed one or more biologics (one biological failure, 20.6%; two biological failures, 27.8%; three biological failures, 16.5%; four biological failures, 10.3%; five biological failures, 1.1%). After 3 months of FIL therapy, all clinimetric index results significantly improved from baseline, as well as after 6 months. Also, ESR and CRP significatively decreased at T3 and T6. Two cases of HZ were recorded, while no new MACE, VTE, or cancer were recorded during the observation time. Conclusion: Despite the limitations of the retrospective study and of the observational period of only 6 months, real-life data on the treatment of RA patients with FIL demonstrate that this Jak inhibitor therapy is safe in terms of CV, VTE events, and occurrence of cancer, and is also effective in a population identified as "difficult to treat" due to failure of previous b-DMARD therapy.
Language	English
Publishing date	2023-08-25
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2662248-8
ISSN	2075-4426
ISSN	2075-4426
DOI	10.3390/jpm13091303
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Article ; Online: ToRaRI (Tofacitinib in Rheumatoid Arthritis a Real-Life experience in Italy): Effectiveness, safety profile of tofacitinib and concordance between patient-reported outcomes and physician's global assessment of disease activity in a retrospective study in Central-Italy.

D'Alessandro, Francesco / Cazzato, Massimiliano / Laurino, Elenia / Morganti, Riccardo / Bardelli, Marco / Frediani, Bruno / Buongarzone, Claudia / Moroncini, Gianluca / Guiducci, Serena / Cometi, Laura / Benucci, Maurizio / Ligobbi, Francesca / Marotto, Daniela / Mosca, Marta

Clinical rheumatology

2023 Volume 43, Issue 2, Page(s) 657–665

Abstract: Introduction: The use of Janus Kinase Inhibitors (JAK-Is) in rheumatoid arthritis (RA) has entered in daily practice. In consideration of ORAL-Surveillance trial and the new EULAR recommendations, real-world data are needed to assess Jak-Is safety and ... ...

Abstract	Introduction: The use of Janus Kinase Inhibitors (JAK-Is) in rheumatoid arthritis (RA) has entered in daily practice. In consideration of ORAL-Surveillance trial and the new EULAR recommendations, real-world data are needed to assess Jak-Is safety and effectiveness. The multicenter study presented here aimed to evaluate effectiveness and safety of tofacitinib in a real-life cohort. Methods: A retrospective analysis was performed from September 2021 to December 2022. Data were collected when tofacitinib was started (T0) and after 3 (T3), 6 (T6) and 12 (T12) months of treatment. The primary objective was to analyze the efficacy and safety of tofacitinib. Safety was assessed by recording adverse events (AEs) with and without discontinuation. The secondary objective was to assess the difference between Patient-Reported Outcomes (PROs) and Physician's Global Assessment of disease activity (PhGA). Results: 122 patients were included in the study from the following rheumatology Centers: Pisa, Ancona, Florence (two Centers), Siena, and Sardinia. A statistically significant improvement in DAS-28-CRP, CDAI and SDAI score was observed at T3, T6, compared to baseline (p < 0.001). Improvement was confirmed in patients who reach T12. Patients naïve to bDMARDs showed a shorter remission time and higher remission rates. There was also a statistically significant improvement in PROs compared to baseline (p < 0.001). The improvement was rapid and was consistent with PhGA. The 12-month retention rate for tofacitinib was 89.35%. Reasons to stop tofacitinib were: insufficient response (7), gastrointestinal symptoms (2), infection (1), malignancy (1), Zoster (1), pruritus sine materia (1). Conclusions: Tofacitinib is safe and effective in our RA cohort. It induces higher remission rates in patients naive to bDMARDs, suggesting that there may be a benefit using it as first-line therapy. Additionally, improvement in PROs was consistent with PhGA scores, demonstrating how tofacitinib affects both the objective and subjective components of disease activity. Key Points 1. JAK inhibitors are considered at a similar level as biologic agents in terms of effectiveness. 2. After ORAL-Surveillance results, real-world data are needed to assess the benefit/risk profile of Jaki. 3. Disagreement between patients and physicians has been previously reported with biologic therapy among patients with rheumatoid arthritis, with patients rating disease activity higher than physicians. 4. Jak inhibitors could reduce this discrepancy, due to their mechanism of action.
MeSH term(s)	Humans ; Antirheumatic Agents/adverse effects ; Retrospective Studies ; Janus Kinase Inhibitors/adverse effects ; Arthritis, Rheumatoid/diagnosis ; Pyrroles/adverse effects ; Patient Reported Outcome Measures ; Treatment Outcome ; Piperidines ; Pyrimidines
Chemical Substances	Antirheumatic Agents ; Janus Kinase Inhibitors ; tofacitinib (87LA6FU830) ; Pyrroles ; Piperidines ; Pyrimidines
Language	English
Publishing date	2023-12-23
Publishing country	Germany
Document type	Multicenter Study ; Journal Article
ZDB-ID	604755-5
ISSN	1434-9949 ; 0770-3198
ISSN (online)	1434-9949
ISSN	0770-3198
DOI	10.1007/s10067-023-06836-w
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Article: Fibrosing Progressive Interstitial Lung Disease in Rheumatoid Arthritis: A Multicentre Italian Study.

Sebastiani, Marco / Venerito, Vincenzo / Laurino, Elenia / Gentileschi, Stefano / Atzeni, Fabiola / Canofari, Claudia / Andrisani, Dario / Cassone, Giulia / Lavista, Marlea / D'Alessandro, Francesco / Vacchi, Caterina / Scardapane, Arnaldo / Frediani, Bruno / Cazzato, Massimiliano / Salvarani, Carlo / Iannone, Florenzo / Manfredi, Andreina

Journal of clinical medicine

2023 Volume 12, Issue 22

Abstract: Background: The INBUILD study demonstrated the efficacy of nintedanib in the treatment of progressive fibrosing interstitial lung disease different to idiopathic pulmonary fibrosis, including rheumatoid arthritis (RA)-related ILD. Nevertheless, the ... ...

Abstract	Background: The INBUILD study demonstrated the efficacy of nintedanib in the treatment of progressive fibrosing interstitial lung disease different to idiopathic pulmonary fibrosis, including rheumatoid arthritis (RA)-related ILD. Nevertheless, the prevalence of RA-ILD patients that may potentially benefit from nintedanib remains unknown. Objectives and methods: The aim of the present multicentre study was to investigate the prevalence and possible associated factors of fibrosing progressive patterns in a cross-sectional cohort of RA-ILD patients. Results: One hundred and thirty-four RA-ILD patients with a diagnosis of RA-ILD, who were confirmed at high-resolution computed tomography and with a follow-up of at least 24 months, were enrolled. The patients were defined as having a progressive fibrosing ILD in case of a relative decline in forced vital capacity > 10% predicted and/or an increased extent of fibrotic changes on chest imaging in a 24-month period. Respiratory symptoms were excluded to reduce possible bias due to the retrospective interpretation of cough and dyspnea. According to radiologic features, ILD was classified as usual interstitial pneumonia (UIP) in 50.7% of patients, nonspecific interstitial pneumonia in 19.4%, and other patterns in 29.8%. Globally, a fibrosing progressive pattern was recorded in 36.6% of patients (48.5% of patients with a fibrosing pattern) with a significant association to the UIP pattern. Conclusion: We observed that more than a third of RA-ILD patients showed a fibrosing progressive pattern and might benefit from antifibrotic treatment. This study shows some limitations, such as the retrospective design. The exclusion of respiratory symptoms' evaluation might underestimate the prevalence of progressive lung disease but increases the value of results.
Language	English
Publishing date	2023-11-11
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2662592-1
ISSN	2077-0383
ISSN	2077-0383
DOI	10.3390/jcm12227041
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Article: Assessing disease activity of rheumatoid arthritis patients and drug-utilization patterns of biologic disease-modifying antirheumatic drugs in the Tuscany region, Italy.

Frontiers in pharmacology

2023 Volume 14, Page(s) 1244486

Abstract: Introduction: ...

Abstract	Introduction:
Language	English
Publishing date	2023-09-25
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2587355-6
ISSN	1663-9812
ISSN	1663-9812
DOI	10.3389/fphar.2023.1244486
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Article ; Online: Dose adjustments and discontinuation in TNF inhibitors treated patients: when and how. A systematic review of literature.

Ruscitti, Piero / Sinigaglia, Luigi / Cazzato, Massimiliano / Grembiale, Rosa Daniela / Triolo, Giovanni / Lubrano, Ennio / Montecucco, Carlomaurizio / Giacomelli, Roberto

Rheumatology (Oxford, England)

2018 Volume 57, Issue 57 Suppl 7, Page(s) vii23–vii31

Abstract: Objectives: To review the available evidence concerning the possibility of discontinuing and/or tapering the dosage of TNF inhibitors (TNFi) in RA patients experiencing clinical remission or low disease activity.: Methods: A systematic review of the ... ...

Abstract	Objectives: To review the available evidence concerning the possibility of discontinuing and/or tapering the dosage of TNF inhibitors (TNFi) in RA patients experiencing clinical remission or low disease activity. Methods: A systematic review of the literature concerning the low dosage and discontinuation of TNFi in disease-controlled RA patients was performed by evaluation of reports published in indexed international journals (Medline via PubMed, EMBASE), in the time frame from 8 April 2013 to 15 January 2016. Results: We analysed the literature evaluating the efficacy and the safety of two different strategies using TNFi, decreasing dosage or discontinuation, in patients experiencing clinical remission or low disease activity. After the analysis of online databases, 25 references were considered potentially relevant and 16 references were selected. The majority of data concerned etanercept and adalimumab. Results suggested the induction of stable clinical remission or low disease activity by using TNFi followed by a dosage tapering and/or discontinuation of such drugs may be associated with the maintenance of a good clinical response in a subset of patients affected by early disease. Conclusion: RA patients treated early with TNFi and achieving their therapeutic clinical targets seem to maintain their clinical response after tapering or discontinuing TNFi. These data may allow physicians a more dynamic and tailored management of RA patients.
MeSH term(s)	Antirheumatic Agents/administration & dosage ; Arthritis, Rheumatoid/drug therapy ; Dose-Response Relationship, Drug ; Humans ; Remission Induction ; Tumor Necrosis Factor-alpha/antagonists & inhibitors
Chemical Substances	Antirheumatic Agents ; Tumor Necrosis Factor-alpha
Language	English
Publishing date	2018-10-05
Publishing country	England
Document type	Journal Article ; Review
ZDB-ID	1464822-2
ISSN	1462-0332 ; 1462-0324
ISSN (online)	1462-0332
ISSN	1462-0324
DOI	10.1093/rheumatology/key132
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Article: Utilisation patterns and clinical impact of the introduction of infliximab-biosimilar in Tuscany, Italy: real world evidence following the recommendation of switching for non-medical reasons.

Convertino, Irma / Lucenteforte, Ersilia / Gini, Rosa / Lorenzoni, Valentina / Cazzato, Massimiliano / Turchetti, Giuseppe / Trieste, Leopoldo / Ferraro, Sara / Leonardi, Luca / Roberto, Giuseppe / Luciano, Nicoletta / Blandizzi, Corrado / Mosca, Marta / Tuccori, Marco

Clinical and experimental rheumatology

2020 Volume 39, Issue 4, Page(s) 753–762

Abstract: Objectives: This study was aimed at assessing the impact of a non-medical recommendation on drug-utilisation patterns and clinical outcomes in a central Region of Italy (Tuscany).: Methods: We performed a pre-post study on data collected in Tuscan ... ...

Abstract	Objectives: This study was aimed at assessing the impact of a non-medical recommendation on drug-utilisation patterns and clinical outcomes in a central Region of Italy (Tuscany). Methods: We performed a pre-post study on data collected in Tuscan healthcare administrative databases. We included patients with diagnosis of rheumatoid arthritis, or psoriatic arthritis, or ankylosing spondylitis, or ulcerative colitis, or Crohn's disease, or psoriasis. The first analysis compared patients treated with infliximab on January 1st, 2013 (originator only available) to those on January 1st, 2016 (both originator and biosimilar available). The second analysis compared infliximab-originator users with infliximab-biosimilar ones. Adjusted odds ratios (OR) of persistence on treatment, Emergency Department (ED) admissions, hospitalisations and specialist visits were calculated. Results: The first analysis included 606 patients and the second 434. In both analyses, we did not observe any significant difference in persistence. In the first analysis, the 2016 infliximab-originator cohort showed a significant association with the risk of having at least one ED admission (OR 1.54, 95% CI 1.02 to 2.31). A significant difference of accessing a specialist visit (more frequently rheumatologic) was observed in the 2016 cohort (OR 1.52, 95% CI 1.05 to 2.20). In the second analysis, the risk of having at least one hospitalisation decreased significantly in switchers to infliximab-biosimilar (OR 0.49, 95% CI 0.26 to 0.96). Conclusions: Our study showed no relevant changes in the clinical outcomes following the introduction of infliximab-biosimilar. The few observed differences observed can be explained mainly by a selective switching to infliximab-biosimilar in patients with lower burden of disease.
MeSH term(s)	Antibodies, Monoclonal/adverse effects ; Biosimilar Pharmaceuticals/adverse effects ; Drug Substitution ; Humans ; Infliximab/adverse effects ; Italy/epidemiology ; Treatment Outcome
Chemical Substances	Antibodies, Monoclonal ; Biosimilar Pharmaceuticals ; Infliximab (B72HH48FLU)
Language	English
Publishing date	2020-08-05
Publishing country	Italy
Document type	Journal Article
ZDB-ID	605886-3
ISSN	1593-098X ; 0392-856X
ISSN (online)	1593-098X
ISSN	0392-856X
DOI	10.55563/clinexprheumatol/pa6lap
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Article: Trajectories of Adherence to Biologic Disease-Modifying Anti-Rheumatic Drugs in Tuscan Administrative Databases: The Pathfinder Study.

Convertino, Irma / Giometto, Sabrina / Gini, Rosa / Cazzato, Massimiliano / Fornili, Marco / Valdiserra, Giulia / Cappello, Emiliano / Ferraro, Sara / Bartolini, Claudia / Paoletti, Olga / Tillati, Silvia / Baglietto, Laura / Turchetti, Giuseppe / Trieste, Leopoldo / Lorenzoni, Valentina / Blandizzi, Corrado / Mosca, Marta / Tuccori, Marco / Lucenteforte, Ersilia

Journal of clinical medicine

2021 Volume 10, Issue 24

Abstract: Scanty information on clustering longitudinal real-world data is available in the medical literature about the adherence implementation phase in rheumatoid arthritis (RA). To identify and characterize trajectories by analyzing the implementation phase of ...

Abstract	Scanty information on clustering longitudinal real-world data is available in the medical literature about the adherence implementation phase in rheumatoid arthritis (RA). To identify and characterize trajectories by analyzing the implementation phase of adherence to biologic Disease-Modifying Anti-Rheumatic Drugs (DMARDs), we conducted a retrospective cohort drug-utilization study using Tuscan administrative databases. RA patients were identified by a validated algorithm, including the first biologic DMARD supply from 2010 to 2015, RA specialist visit in the year before or after the first supply date and RA diagnosis in the five years before or in the year after the first supply date. We observed users for three years or until death, neoplasia, or pregnancy. We evaluated adherence quarterly through the Medication Possession Ratio. Firstly, we identified adherence trajectories and described the baseline characteristics; then, we focused on the trajectory most populated to distinguish the related sub-trajectories. We identified 952 first ever-biologic DMARD users in RA (712 females, mean age 52.7 years old, standard deviation 18.8). The biologic DMARD mostly supplied was etanercept (387 users) followed by adalimumab (233). Among 935 users with at least 3 adherence values, we identified 49 fully-adherent users, 829 continuous users, and 57 early-discontinuing users. Significant differences were observed among the index drugs. After focusing on the continuous users, three sub-trajectories were identified: continuous-steady users (556), continuous-alternate users (207), and continuous-declining users (66). No relevant differences emerged at the baseline. The majority of first ever-biologic DMARD users showed a continuous adherence behavior in RA. The role of adherence potential predictors and the association with effectiveness and safety outcomes should be explored by further studies.
Language	English
Publishing date	2021-12-08
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2662592-1
ISSN	2077-0383
ISSN	2077-0383
DOI	10.3390/jcm10245743
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Article ; Online: Validation of algorithms for selecting rheumatoid arthritis patients in the Tuscan healthcare administrative databases.

Convertino, Irma / Cazzato, Massimiliano / Giometto, Sabrina / Gini, Rosa / Valdiserra, Giulia / Cappello, Emiliano / Ferraro, Sara / Tillati, Silvia / Bartolini, Claudia / Paoletti, Olga / Lorenzoni, Valentina / Trieste, Leopoldo / Filippi, Matteo / Turchetti, Giuseppe / Cristofano, Michele / Blandizzi, Corrado / Mosca, Marta / Lucenteforte, Ersilia / Tuccori, Marco

Scientific reports

2021 Volume 11, Issue 1, Page(s) 20314

Abstract: Validation of algorithms for selecting patients from healthcare administrative databases (HAD) is recommended. This PATHFINDER study section is aimed at testing algorithms to select rheumatoid arthritis (RA) patients from Tuscan HAD (THAD) and assessing ... ...

Abstract	Validation of algorithms for selecting patients from healthcare administrative databases (HAD) is recommended. This PATHFINDER study section is aimed at testing algorithms to select rheumatoid arthritis (RA) patients from Tuscan HAD (THAD) and assessing RA diagnosis time interval between the medical chart date and that of THAD. A population was extracted from THAD. The information of the medical charts at the Rheumatology Unit of Pisa University Hospital represented the reference. We included first ever users of biologic disease modifying anti-rheumatic drugs (bDMARDs) between 2014 and 2016 (index date) with at least a specialist visit at the Rheumatology Unit of the Pisa University Hospital recorded from 2013 to the index date. Out of these, we tested four index tests (algorithms): (1) RA according to hospital discharge records or emergency department admissions (ICD-9 code, 714*); (2) RA according to exemption code from co-payment (006); (3) RA according to hospital discharge records or emergency department admissions AND RA according to exemption code from co-payment; (4) RA according to hospital discharge records or emergency department admissions OR RA according to exemption code from co-payment. We estimated sensitivity, specificity, positive and negative predicted values (PPV and NPV) with 95% confidence interval (95% CI) and the RA diagnosis median time interval (interquartile range, IQR). Two sensitivity analyses were performed. Among 277 reference patients, 103 had RA. The fourth algorithm identified 96 true RA patients, PPV 0.78 (95% CI 0.70-0.85), sensitivity 0.93 (95% CI 0.86-0.97), specificity 0.84 (95% CI 0.78-0.90), and NPV 0.95 (95% CI 0.91-0.98). The sensitivity analyses confirmed performance. The time measured between the actual RA diagnosis date recorded in medical charts and that assumed in THAD was 2.2 years (IQR 0.5-8.4). In conclusion, this validation showed the fourth algorithm as the best. The time interval elapsed between the actual RA diagnosis date in medical charts and that extrapolated from THAD has to be considered in the design of future studies.
MeSH term(s)	Adult ; Aged ; Algorithms ; Antirheumatic Agents/therapeutic use ; Arthritis, Rheumatoid/epidemiology ; Data Management ; Databases, Factual ; Female ; Hospitals ; Humans ; International Classification of Diseases ; Italy/epidemiology ; Male ; Middle Aged ; Patient Admission ; Patient Discharge ; Patient Selection ; Predictive Value of Tests ; Reproducibility of Results ; Retrospective Studies ; Rheumatology/methods ; Sensitivity and Specificity
Chemical Substances	Antirheumatic Agents
Language	English
Publishing date	2021-10-13
Publishing country	England
Document type	Journal Article ; Validation Study
ZDB-ID	2615211-3
ISSN	2045-2322 ; 2045-2322
ISSN (online)	2045-2322
ISSN	2045-2322
DOI	10.1038/s41598-021-98321-0
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Article ; Online: A case of adult periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome associated with endocapillary proliferative glomerulonephritis.

Cazzato, Massimiliano / Neri, Rossella / Possemato, Niccolo / Puccini, Rodolfo / Bombardieri, Stefano

Clinical rheumatology

2013 Volume 32 Suppl 1, Page(s) S33–6

Abstract: PFAPA is an acronym for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis. This syndrome has been usually described in pediatric patients and it generally resolves spontaneously. The endocapillary proliferative glomerulonephritis ( ... ...

Abstract	PFAPA is an acronym for periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis. This syndrome has been usually described in pediatric patients and it generally resolves spontaneously. The endocapillary proliferative glomerulonephritis (EPG) is a glomerular injury characterized by hypercellularity in glomerular lumen and is caused by post-infectious or autoimmune diseases. In this paper, we describe the case of a 35-year-old man affected by PFAPA and EPG. To our knowledge this association has never been reported in the literature before.
MeSH term(s)	Adult ; Dose-Response Relationship, Drug ; Fever/diagnosis ; Glomerulonephritis, Membranoproliferative/diagnosis ; Glomerulonephritis, Membranoproliferative/drug therapy ; Glucocorticoids/therapeutic use ; Humans ; Lymph Nodes/pathology ; Lymphadenitis/diagnosis ; Male ; Methylprednisolone/therapeutic use ; Neck ; Periodicity ; Pharyngitis/diagnosis ; Pulse Therapy, Drug ; Stomatitis, Aphthous/diagnosis ; Syndrome ; Treatment Outcome
Chemical Substances	Glucocorticoids ; Methylprednisolone (X4W7ZR7023)
Language	English
Publishing date	2013-03
Publishing country	Germany
Document type	Case Reports ; Journal Article
ZDB-ID	604755-5
ISSN	1434-9949 ; 0770-3198
ISSN (online)	1434-9949
ISSN	0770-3198
DOI	10.1007/s10067-010-1420-8
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