Primary Sjögren's Syndrome: Extraglandular Manifestations and Hydroxychloroquine Therapy

Julia Demarchi¹, Silvia Beatriz Papasidero², María Alejandra Medina³, Diana Klajn⁴, Rafael Chaparro del Moral², Oscar Luis Rillo⁵, María Victoria Martire⁶, Gloria Crespo⁷, Anastasia Secco⁸, Antonio Catalan Pellet⁶, Cristina Amitrano⁹, Catherine Crow¹⁰, Cecilia Asnal⁹, Paula Pucci⁹, Francisco Caeiro¹¹, Nadia Benzaquén¹¹, Juan Pablo Pirola¹¹, Marcela Colazo¹¹, M. Mayer¹², F. Zazzetti¹², S. Velez¹², J. C. Barreira¹³, Natalia Tamborenea¹⁴, M. L. Santiago¹⁴ and Laura Raiti¹⁵, ¹Rheumatology Deparment, Hospital General de Agudos Dr. Enrique Tornú, Buenos Aires, Argentina, ²Rheumatology Department, Rheumatology Unit, Hospital General de Agudos Dr. E. Tornú, Buenos Aires, Argentina, ³Rheumatology Department, Hospital General de Agudos Dr. Enrique Tornú, Buenos Aires, Argentina, ⁴Research Committee, Research Committee, Hospital General de Agudos Dr. E. Tornú, Buenos Aires, Argentina, ⁵Rheumatology Department, Hospital General de Agudos “Dr. Ignacio Pirovano”, Buenos Aires, Argentina, Buenos Aires, Argentina, ⁶Rheumatology, Hospital Bernardino Rivadavia, Buenos Aires, Argentina, ⁷Hospital Bernardino Rivadavia, Ciudad Autónoma de Buenos Aires, Argentina, ⁸Hospital Bernardino Rivadavia, Buenos Aires, Argentina, ⁹Rheumatology, Hospital Alemán, Buenos Aires, Argentina, ¹⁰Rheumatology, Hospital Alemán, Buenos aires, Argentina, ¹¹Rheumatology, Hospital Privado Centro Médico de Córdoba, Córdoba, Argentina, ¹²Hospital Británico, Buenos Aires, Argentina, ¹³Rheumatology Service, Hospital Británico, Buenos Aires, Buenos Aires, Argentina, ¹⁴Rheumatology, OMI, Buenos Aires, Argentina, ¹⁵Clínica Bessone, San Miguel, Argentina

Meeting: 2016 ACR/ARHP Annual Meeting

Date of first publication: September 28, 2016

Keywords: Sjogren's syndrome and hydroxychloroquine

Session Information

Date: Tuesday, November 15, 2016

Title: Sjögren's Syndrome - Poster II: Clinical Science

Session Type: ACR Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose: The use of Hydroxychloroquine (HCQ) in Primary Sjögren’s Syndrome (PSS) has been assessed in different studies over the last years, with conflicting results regarding its efficacy in SICCA syndrome and extraglandular manifestations (EGM). The purpose of this study was to compare the incidence rate of EGM in PSS patients with and without HCQ therapy.

Methods: We performed a multicenter retrospective study, including patients with PSS (European Classification Criteria) with at least one year of follow-up. Subjects with concomitant fibromyalgia, autoimmune hepatitis, primary biliary cirrhosis and primary sclerosing cholangitis were excluded. Demographics and PSS characteristics were recorded. The EGM were defined by EULAR SS Disease Activity Index (ESSDAI). Patients were divided into 2 groups according to their use or not of HCQ therapy. We evaluated the use of HCQ and its relationship to EGM. HCQ therapy was defined as the continuous use of the drug for at least 3 months. Statistical analysis: a descriptive analysis of demographics and PSS characteristics was performed. We compared the incidence of EGM between groups defined by HCQ therapy using chi² test or Fisher’s exact test.

Results: A total of 221 patients were included (97.3% women), mean age: 55.7 years (SD, 14). Mean age at diagnosis: 48.8 years (SD, 15). Median disease duration: 60 months (IQR 35-84). One hundred and seventy patients (77%) received HCQ. About half of the patients had at least one EGM during the course of the disease, 20% of them developed an EGM before the onset of the SICCA syndrome and 26% simultaneously with dryness symptom. Overall, EGM were less frequent in those on HCQ therapy (36.5% vs 63.5%, p<0,001). Considering each EGM individually, the following manifestations were more frequent in the non-treated group: arthritis (p<0,001), fatigue (p< 0,001), purpure (p=0,01), Raynaud phenomenon (p= 0,003) and hypergammaglobulinemia (p=0,006). (Table I) Immunosuppressive treatment was indicated on 28 patients (12,7%), 13 of which were receiving also HCQ . The first reason for those treatments was the presence of arthritis in 12/28 patients (42.8 %), and the drug used in all the cases was methotrexate. Only three patients required immunosuppressive therapy with cyclophosphamide, due to the presence of glomerulonephritis, vasculitis and interstitial lung disease. None of the patients received biologic therapy.

Conclusion: The lower incidence of EGM observed in patients on HCQ therapy supports its efficacy in PSS. However, further large scale prospective studies are needed to confirm these findings.

Disclosure: J. Demarchi, None; S. B. Papasidero, None; M. A. Medina, None; D. Klajn, None; R. Chaparro del Moral, None; O. L. Rillo, None; M. V. Martire, None; G. Crespo, None; A. Secco, None; A. Catalan Pellet, None; C. Amitrano, None; C. Crow, None; C. Asnal, None; P. Pucci, None; F. Caeiro, None; N. Benzaquén, None; J. P. Pirola, None; M. Colazo, None; M. Mayer, None; F. Zazzetti, None; S. Velez, None; J. C. Barreira, None; N. Tamborenea, None; M. L. Santiago, None; L. Raiti, None.

To cite this abstract in AMA style:

Demarchi J, Papasidero SB, Medina MA, Klajn D, Chaparro del Moral R, Rillo OL, Martire MV, Crespo G, Secco A, Catalan Pellet A, Amitrano C, Crow C, Asnal C, Pucci P, Caeiro F, Benzaquén N, Pirola JP, Colazo M, Mayer M, Zazzetti F, Velez S, Barreira JC, Tamborenea N, Santiago ML, Raiti L. Primary Sjögren’s Syndrome: Extraglandular Manifestations and Hydroxychloroquine Therapy [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/primary-sjogrens-syndrome-extraglandular-manifestations-and-hydroxychloroquine-therapy/. Accessed .

« Back to 2016 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/primary-sjogrens-syndrome-extraglandular-manifestations-and-hydroxychloroquine-therapy/