Palindromic rheumatism
Summary
The summary that appears below is taken from the Autoimmune Association. As you can see from the below quote, the Autoimmune Association attributes the information to the Genetic and Rare Diseases Information Center. The Genetic and Rare Diseases Information Center’s entry on Palindromic rheumatism is in unattributed quotes, and is not as comprehensive as what appears on the Autoimmune Association’s website. That’s why I chose to include the information from the Autoimmune Association instead of what currently appears on the Genetic and Rare Diseases Information site.
Palindromic rheumatism (PR) is an autoimmune related disease characterized by sudden, multiple, and recurring attacks of joint pain and swelling, typically in the hands and feet. Each episode may last from several hours to several days. The frequency of attacks also varies, from one episode a day to several during the course of a year. Between attacks, the symptoms disappear and the affected joints appear normal on x-ray exams. The cause of palindromic rheumatism is unknown, although a possible allergic origin has been suggested. There may also be a clinical association between antiphospholipid syndrome and palindromic rheumatism. Some individuals with palindromic rheumatism develop chronic joint inflammation and go on to develop rheumatoid arthritis.
Palindromic rheumatism (PR) is a type of recurrent arthritis characterized by episodes or “attacks” of joint inflammation, sequentially affecting one to several joint areas for hours to days.[1][2] A PR attack often occurs suddenly without any obvious triggers or warning symptoms. Any joint(s) may be affected, but finger joints, wrists, and knees are most commonly affected.[2] Symptoms during episodes may include pain, swelling, stiffness, and redness in and around the joints.[2] Some people may have a fever and other systemic symptoms.[3] Between episodes, people with PR have no symptoms.[2] The time between episodes may last from days to months.[1]
The underlying cause of PR is not known.[2] Some people with PR eventually develop chronic rheumatic disease, the most common being rheumatoid arthritis (RA). Others may develop lupus and/or other systemic disorders. People with anti-CCP antibodies detected in a blood test appear more likely to develop RA.[1] There has been some evidence to support that PR can be a presenting feature of RA (or part of the spectrum of RA), rather than a distinct condition.[2] [4]
Treatment of PR is challenging, as it has not been studied in randomized trials.[5] Nonsteroidal anti-inflammatory drugs (NSAIDs) may be used to treat acute episodes. Disease-modifying antirheumatic drugs (DMARDs) may be an option for those with refractory, frequent attacks.[5] Hydroxychloroquine (a DMARD) reportedly may help control attacks and reduce the risk of progression to RA.[2][4]
This information is provided by the National Institutes of Health (NIH) Genetic and Rare Diseases Information Center (GARD).
https://rarediseases.info.nih.gov/diseases/7304/palindromic-rheumatism(Autoimmune Association, 2023)
Palindromic rheumatism is a type of arthritis characterized by episodes of joint pain flare-ups, which fortunately do not cause any lasting damage to joints. The attacks that people experience can last for several days and travel from joint to joint.
(Global Autoimmune Institute, 2023)
Symptoms
Painful and stiff joints that may feel tender and hot, redness on the skin over the joints, fatigue.
(Global Autoimmune Institute, 2023)
Diagnostic Criteria
There is no consensus diagnostic criteria for palindromic rheumatism. There are four proposed diagnostic criteria listed in the table below:
(Sanmartí et. al, 2021)
Study Classification Criteria
Variable.
Diagnostic Tests
Non-specific to a diagnosis of Palindromic rheumatism:
Rheumatoid factor (RF)
Antinuclear antibodies (ANA)
C-Reactive Protein (CRP)
Estimated sedimentation rate (ESR)
uricemia (measures the levels of uric acid in the blood)
Anti–citrullinated protein antibody (ANCPA)
anti-carbamylated protein antibodies (anti-CarP) (found in a quarter of longstanding patients with Palindromic rheumatism)
(Sanmarti et. al, 2021)
Organized Autoimmunity
(Alternative Autoimmune Disease Classification: FIEM, MIEM or BIEM, or FEM, MEM or BEM)
sex predominance (is an autoimmune disease primarily found in genetic Females, Males, or equally in Both?)
More research needed.
Inherited and acquired gene variations that cause increased susceptibility
Human Leukocyte Antigen (HLA) Associations
More research needed.
Other Gene Variations (mutations)
Mediterranean Fever (MEFV) variations (Cuervo et. al, 2021)
Gene Triggering Environmental Exposures
Infections
More research needed.
Toxins
More research needed.
Stress
Needs to be assessed for each patient
Multiple interactive and destructive immune system pathologies
More research needed.
Tissue-Type or Cell-Type Attacked
Tissues around the joint(s) become inflamed, known as “periarticular” inflammation. Some research shows that flares may cause synovial inflammation, but between flares, joint ultrasound reveals no lasting damage/inflammation. (Sanmarti et. al, 2021)
Treatment(s)
No randomized clinical trials have been conducted (Sanmarti et. al, 2021).
Observational studies and case series have found:
treatment with NSAIDs {non-steroidal anti-inflammatory drugs} produces variable results
anecdotal reports of effective treatment with glucocorticoids
anti-malarials (such as hydroxychloroquine) show some promise in reducing the frequency and severity of flare ups
“Methotrexate, the most widely-used drug in the treatment of RA {Rheumatoid arthritis}, has not been tested in PR, although a recent observational, uncontrolled study used methotrexate as part of a treat-to-target strategy that resulted in substantial clinical benefits, with low rates of progression to RA”
“A surprisingly high rate of clinical response was observed with rituximab in an Indian survey”
“Colchicine is effective in seronegative {antibody-negative} PR {Palindromic rheumatism} patients with MEFV {Mediterranean Fever} mutations”
(Sanmarti et. al, 2021)
Managing Specialist(s)
Rheumatologist
Research Authors
Canada
L. Ellingwood, University of Western Ontario, London Ontario, Canada
O. Schieir, University of Toronto, Toronto Ontario, Canada
M. F. Valois, McGill University, Montreal Quebec, Canada
S. J. Bartlett, McGill University, Montreal Quebec, Canada
L. Bessette, CHU de Québec‐Université Laval, Laval Quebec, Canada
G. Boire, Centre intégré universitaire de santé et de services sociaux de l'Estrie – Centre hospitalier universitaire de Sherbrooke (CIUSSS de l'Estrie‐CHUS) and Université de Sherbrooke
G. Hazlewood, University of Calgary, Calgary Alberta, Canada
C. Hitchon, University of Manitoba, Winnipeg Manitoba, Canada
E. C. Keystone, Mount Sinai Hospital and University of Toronto, Toronto Ontario, Canada
D. Tin, Southlake Regional Health Centre, Newmarket Ontario, Canada
C. Thorne, Southlake Regional Health Centre, Newmarket Ontario, Canada
V. P. Bykerk, Hospital for Special Surgery, Weill Cornell Medical College, New York, New York, and University of Toronto, Toronto Ontario, Canada
V. P. Bykerk, Hospital for Special Surgery, Weill Cornell Medical College, New York, New York, and University of Toronto, Toronto Ontario, Canada
Paraguay
Sonia Cabrera-Villalba, Hospital Central del Instituto de Previsión Social, Asunción, Paraguay and Hospital de Clínicas Universidad Nacional de Asunción, Asunción, Paraguay
Spain
Raul Castellanos-Moreira, Arthritis Unit, Rheumatology Department, Hospital Clinic of Barcelona
S.C. Rodriguez-Garcia, MD, Arthritis Unit, Rheumatology Department, Hospital Clinic of Barcelona
J.A. Gómez-Puerta, MD, PhD, MPH, Arthritis Unit, Rheumatology Department, Hospital Clinic of Barcelona
V. Ruiz-Esquide, MD, PhD, Arthritis Unit, Rheumatology Department, Hospital Clinic of Barcelona
O. Camacho, MD, Arthritis Unit, Rheumatology Department, Hospital Clinic of Barcelona
J. Ramírez, MD, PhD, Arthritis Unit, Rheumatology Department, Hospital Clinic of Barcelona
A. Cuervo, MD, Arthritis Unit, Rheumatology Department, Hospital Clinic of Barcelona
R. Morlà, MD, PhD, Arthritis Unit, Rheumatology Department, Hospital Clinic of Barcelona
J.D. Cañete, MD, PhD, Arthritis Unit, Rheumatology Department, Hospital Clinic of Barcelona
I. Haro, PhD, Unit of Synthesis and Biomedical Applications of Peptides, IQAC-CSIC
R. Sanmarti, MD, PhD, Arthritis Unit, Rheumatology Department, Hospital Clinic of Barcelona.
Beatriz Frade-Sosa, Arthritis Unit, Rheumatology Department, Hospital Clinic of Barcelona
Georgina Salvador, Rheumatology Department, University Hospital Mutua Tarrasa, Barcelona
Research Institutions
Canada
University of Western Ontario, London Ontario
University of Toronto, Toronto Ontario
McGill University, Montreal Quebec
CHU de Québec‐Université Laval, Laval Quebec
Centre intégré universitaire de santé et de services sociaux de l'Estrie – Centre hospitalier universitaire de Sherbrooke (CIUSSS de l'Estrie‐CHUS) and Université de Sherbrooke
University of Calgary, Calgary Alberta
University of Manitoba, Winnipeg Manitoba
Southlake Regional Health Centre, Newmarket Ontario
Paraguay
Hospital Central del Instituto de Previsión Social, Asunción, Paraguay
Hospital de Clínicas Universidad Nacional de Asunción, Asunción, Paraguay
Spain
Arthritis Unit, Rheumatology Department, Hospital Clinic of Barcelona
Unit of Synthesis and Biomedical Applications of Peptides, Institute of Advanced Chemistry of Catalonia (IQAC-CSIC), Barcelona
Rheumatology Department, University Hospital Mutua Tarrasa, Barcelona
United States
Mount Sinai Hospital, New York, New York
Hospital for Special Surgery, Weill Cornell Medical College, New York, New York
Average Time from Symptom Onset to Diagnosis
More research needed.
Last Updated
June 23, 2023
References
Cuervo A, Sanmartí R, Ramírez J, Castellanos-Moreira R, Inciarte-Mundo J, Aróstegui JI, McGonagle D, Cañete JD. Palindromic rheumatism: Evidence of four subtypes of palindromic-like arthritis based in either MEFV or rheumatoid factor/ACPA status. Joint Bone Spine. 2021 Dec;88(6):105235. doi: 10.1016/j.jbspin.2021.105235. Epub 2021 Jun 5. PMID: 34098104.
Palindromic rheumatism. Autoimmune Association. (2023). Retrieved June 15, 2023, from https://autoimmune.org/disease-information/palindromic-rheumatism-pr/
Palindromic rheumatism. Global Autoimmune Institute. (2023). Retrieved June 15, 2023, from https://www.autoimmuneinstitute.org/autoimmune-resources/autoimmune-diseases-list/palindromic-rheumatism/
Sanmartí R, Frade-Sosa B, Morlà R, Castellanos-Moreira R, Cabrera-Villalba S, Ramirez J, Salvador G, Haro I, Cañete JD. Palindromic Rheumatism: Just a Pre-rheumatoid Stage or Something Else? Front Med (Lausanne). 2021 Mar 25;8:657983. doi: 10.3389/fmed.2021.657983. PMID: 33842513; PMCID: PMC8026891.