Patients With Co-occurrence of ANCA Vasculitis and Sjögren Syndrome

Patients With Co-occurrence of ANCA Vasculitis and Sjögren Syndrome : a Case Series and Systematic Review

ANCA vasculitis and Sjögren syndrome are two rare diseases, and even more rarely associated. These two conditions have specific organ involvements, and specific follow-up.

The Investigators hypothesise that patients with co-occurrence of these two diseases may have a singular clinical course.

Study Overview

• Status: Completed
• Conditions: Sjogren's Syndrome; ANCA Associated Vasculitis

• Study Type: Observational
• Enrollment (Actual): 5

Contacts and Locations

Study Locations

• France
  • Montpellier, France, 34295
    • UHMontpellier

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

Patients treated in internal medicine services in secondary care centers of Languedoc-Roussillon region.

Description

Inclusion criteria:

• Diagnosis of ANCA vasculitis based on clinical, immunological and histopathological features.
• Diagnosis of Sjögren syndrom based on ACR/EULAR 2016 criteria.

Exclusion criteria:

• Age < 18 years
• Insuffisant data during follow-up

Study Plan

How is the study designed?

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Severity of Sjögren disease	Severity of Sjögren disease	day 1 (Upon diagnosis)

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Sjögren extra-glandular complications	Sjögren extra-glandular complications	day 1 (Upon diagnosis)
severity of ANCA vasculitis	severity of ANCA vasculitis	day 1 (Upon diagnosis)

Collaborators and Investigators

• Sponsor: University Hospital, Montpellier

Publications and helpful links

General Publications

• Guellec D, Cornec-Le Gall E, Groh M, Hachulla E, Karras A, Charles P, Dunogue B, Abad S, Alvarez F, Gerard F, Devauchelle-Pensec V, Pers JO, Puechal X, Guillevin L, Saraux A, Cornec D; CRI (Club Rhumatismes et Inflammation) and the French Vasculitis Study Group. ANCA-associated vasculitis in patients with primary Sjogren's syndrome: detailed analysis of 7 new cases and systematic literature review. Autoimmun Rev. 2015 Aug;14(8):742-50. doi: 10.1016/j.autrev.2015.04.009. Epub 2015 Apr 24.
• Lee SB, Choi H, Kim MK, Jung SM, Song JJ, Park YB, Lee SW. Can antineutrophil cytoplasmic antibody positivity at diagnosis predict the poor outcomes of Sjogren's syndrome? Rheumatol Int. 2020 Jul;40(7):1063-1070. doi: 10.1007/s00296-019-04476-5. Epub 2019 Nov 12.
• Ramos-Casals M, Brito-Zeron P, Font J. The overlap of Sjogren's syndrome with other systemic autoimmune diseases. Semin Arthritis Rheum. 2007 Feb;36(4):246-55. doi: 10.1016/j.semarthrit.2006.08.007. Epub 2006 Sep 22.

Study record dates

Study Major Dates

• Study Start (Actual): May 1, 2021
• Primary Completion (Actual): August 1, 2021
• Study Completion (Actual): September 1, 2021

Study Registration Dates

• First Submitted: May 10, 2021
• First Submitted That Met QC Criteria: May 18, 2021
• First Posted (Actual): May 20, 2021

Study Record Updates

• Last Update Posted (Actual): September 14, 2021
• Last Update Submitted That Met QC Criteria: September 6, 2021
• Last Verified: September 1, 2021

More Information

Terms related to this study

• Keywords: Overlap syndrom
• Additional Relevant MeSH Terms: Pathologic Processes; Cardiovascular Diseases; Vascular Diseases; Immune System Diseases; Autoimmune Diseases; Eye Diseases; Disease; Joint Diseases; Musculoskeletal Diseases; Rheumatic Diseases; Connective Tissue Diseases; Arthritis; Stomatognathic Diseases; Mouth Diseases; Lacrimal Apparatus Diseases; Arthritis, Rheumatoid; Systemic Vasculitis; Xerostomia; Salivary Gland Diseases; Dry Eye Syndromes; Syndrome; Vasculitis; Sjogren's Syndrome; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis
• Other Study ID Numbers: RECHMPL21_0288

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: UNDECIDED
• IPD Plan Description: NC

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No