Palindromic rheumatism: Evidence of four subtypes of palindromic-like arthritis based in either MEFV or rheumatoid factor/ACPA status.

Abstract

INTRODUCTION:

MEFV mutations have been documented in patients with palindromic rheumatism (PR) who do not meet FMF criteria, and RF and ACPA positive RA may start with PR.

OBJECTIVE:

To analyze the clinical phenotype and disease evolution of patients with intermittent, palindromic-like (PL) arthritis seen in our Arthritis Unit according to the RF, ACPA and MEFV mutation status.

METHODS:

MEFV genotyping was done in 76 patients with PL arthritis as defined by predominantly short attacks (≤7days) and a relapsing course. Characteristics of arthritic episodes, RF and ACPA positivity, and the colchicine response were retrospectively collected. Patients were stratified and evaluated according to MEFV mutations and/or positive autoantibodies (ACPA and/or RF).

RESULTS:

Among the patients, 26.3% (20/76) had a MEFV mutation and 23 (30%) were ACPA and/or RF positive. MEFV mutations and/or autoantibody status allowed four PL arthritis patients to be distinguished: group I (MEFV+), with younger age of onset, short duration attacks (<3days), mainly located in the knee, more frequent non-articular manifestations (fever, pericarditis or abdominal pain) and good response to colchicine; group II (autoantibody+) is older than group I, with the same frequency of short attacks, but the most affected joints were the wrists and small joints of hands: 48% met RA classification criteria during follow-up and were taking DMARDs; group III (MEFV- and autoantibody-) was the most frequent (48%) and clinically heterogeneous group; 51% had attacks lasting>3days, and 15 patients developed criteria of immune-mediated inflammatory, autoinflammatory or infectious diseases. Group IV (MEFV+ associated with preexisting immune-inflammatory disease), was associated with very short attacks, like groups I and II, superimposed or coincident with definite immune-inflammatory disease, including seropositive RA, with good response to colchicine.

CONCLUSIONS:

Patients with PL arthritis can be classified in four groups according to the presence or not of MEFV mutations and ACPA/RF antibodies with a different clinical evolution and therapeutic response.

• Authors: Andrea Cuervo, Raimon Sanmartí, Julio Ramírez, Raúl Castellanos-Moreira, José Inciarte-Mundo, Juan I Aróstegui, Dennis McGonagle, Juan D Cañete
• Journal: Joint bone spine (Joint Bone Spine) Vol. 88 Issue 6 Pg. 105235 (12 2021) ISSN: 1778-7254 [Electronic] France
• PMID: 34098104 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: Copyright © 2021 Société française de rhumatologie. Published by Elsevier Masson SAS. All rights reserved.

Chemical References

• Antirheumatic Agents
• Autoantibodies
• MEFV protein, human
• Pyrin
• Rheumatoid Factor

Topics

• Antirheumatic Agents (therapeutic use)
• Arthritis, Rheumatoid (diagnosis, drug therapy, genetics)
• Autoantibodies
• Humans
• Pyrin (genetics)
• Retrospective Studies
• Rheumatoid Factor