The Italian Society of Rheumatology clinical practice guidelines for the management of large vessel vasculitis
N. Ughi,
R. Padoan,
C. Crotti,
S. Sciascia,
G. Carrara,
A. Zanetti,
D. Rozza,
S. Monti,
D. Camellino,
F. Muratore,
G. Emmi,
L. Quartuccio,
S. Morbelli,
K. El Aoufy,
S. Tonolo,
R. Caporali,
S. De Vita,
C. Salvarani,
M.A. Cimmino
Affiliations
N. Ughi
Division of Rheumatology, Multispecialist Medical Department, ASST Grande Ospedale Metropolitano Niguarda, Milan, Italy; Epidemiology Research Unit, SIR, Società Italiana di Reumatologia, Milan
R. Padoan
Rheumatology Unit, Department of Medicine DIMED, Università di Padova, Padua
C. Crotti
Division of Clinical Rheumatology, ASST Pini-CTO, Milan
S. Sciascia
Department of Clinical and Biological Sciences, Center of Research of Immunopathology and Rare Diseases and SCDU Nephrology and Dialysis, Università di Torino, Turin
G. Carrara
Epidemiology Research Unit, SIR, Società Italiana di Reumatologia, Milan
A. Zanetti
Epidemiology Research Unit, SIR, Società Italiana di Reumatologia, Milan, Italy; Department of Statistics and Quantitative Methods, Division of Biostatistics, Epidemiology and Public Health, Università di Milano-Bicocca, Milan
D. Rozza
Epidemiology Research Unit, SIR, Società Italiana di Reumatologia, Milan
S. Monti
Rheumatology Department, IRCCS Policlinico S. Matteo Fondazione, Università di Pavia, Pavia
D. Camellino
Division of Rheumatology, ‘La Colletta’ Hospital, Local Health Trust 3, Arenzano (GE)
F. Muratore
Rheumatology Unit, Azienda Unità Sanitaria Locale-IRCCS di Reggio Emilia, Reggio Emilia
G. Emmi
Department of Experimental and Clinical Medicine, Ospedale Universitario Careggi, Florence
L. Quartuccio
Rheumatology Clinic, Department of Medicine, Università di Udine, ASUFC, Udine
S. Morbelli
IRCCS Ospedale Policlinico San Martino, Genoa
K. El Aoufy
Department of Experimental and Clinical Medicine, Division of Rheumatology, Università di Firenze, Florence, Italy; Forum Italiano dei Professionisti Sanitari in Reumatologia (ForRheuma; Italian Forum of the Health Professionals in Rheumatology), Milan
S. Tonolo
Associazione Nazionale Malati Reumatici (ANMAR; National Association of Rheumatic Patients)
R. Caporali
Division of Clinical Rheumatology, ASST Pini-CTO, Milan, Italy; Department of Clinical Sciences and Community Health, Research Center for Adult and Pediatric Rheumatic Diseases, Research Center for Environmental Health, Università degli Studi di Milano, Milan
S. De Vita
Rheumatology Clinic, Department of Medicine, Università di Udine, ASUFC, Udine
C. Salvarani
Rheumatology Clinic, Department of Medicine, Università di Udine, ASUFC, Udine
M.A. Cimmino
Research Laboratory and Academic Division of Clinical Rheumatology, Department of Internal Medicine, Università di Genova, Genoa
Objective: Since of the last publication of last recommendations on primary large-vessel vasculitis (LVV) endorsed by the Italian Society of Rheumatology (SIR) in 2012, new evidence emerged regarding the diagnosis and the treatment with conventional and biologic immunosuppressive drugs. The associated potential change of clinical care supported the need to update the original recommendations. Methods: Using the grading of recommendations assessment, development and evaluation (GRADE)-ADOLOPMENT framework, a systematic literature review was performed to update the evidence supporting the European Alliance of Associations for Rheumatology (EULAR) guidelines on LVV as reference. A multidisciplinary panel of 12 expert clinicians, a trained nurse, and a patients’ representative discussed the recommendation in cooperation with an Evidence Review Team. Sixty-one stakeholders were consulted to externally review and rate the recommendations. Results: Twelve recommendations were formulated. A suspected diagnosis of LVV should be confirmed by imaging or histology. In active GCA or TAK, the prompt commencement of high dose of oral glucocorticoids (40-60 mg prednisone-equivalent per day) is strongly recommended to induce clinical remission. In selected patients with GCA (e.g., refractory or relapsing disease or patients at risk of glucocorticoid related adverse effects) the use of an adjunctive therapy (tocilizumab or methotrexate) is recommended. In all patients diagnosed with TAK, adjunctive therapies, such as conventional synthetic or biological immunosuppressants, should be given in combination with glucocorticoids. Conclusions: The new set of SIR recommendations was formulated in order to provide a guidance on both diagnosis and treatment of patients suspected of or with a definite diagnosis of LVV.
