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Palindromic rheumatism (PR) is a syndrome characterised by recurrent, self-resolving inflammatory attacks in and around the joints, consists of
arthritis Arthritis is a term often used to mean any disorder that affects joints. Symptoms generally include joint pain and stiffness. Other symptoms may include redness, warmth, swelling, and decreased range of motion of the affected joints. In som ...
or periarticular soft tissue inflammation. The course is often acute onset, with sudden and rapidly developing attacks or flares. There is pain, redness, swelling, and
disability Disability is the experience of any condition that makes it more difficult for a person to do certain activities or have equitable access within a given society. Disabilities may be Cognitive disability, cognitive, Developmental disability, dev ...
of one or multiple joints. The interval between recurrent
palindromic A palindrome is a word, number, phrase, or other sequence of symbols that reads the same backwards as forwards, such as the words ''madam'' or ''racecar'', the date and time ''11/11/11 11:11,'' and the sentence: "A man, a plan, a canal – Panam ...
attacks and the length of an attack is extremely variable from few hours to days. Attacks may become more frequent with time but there is no joint damage after attacks. It is thought to be an
autoimmune disease An autoimmune disease is a condition arising from an abnormal immune response to a functioning body part. At least 80 types of autoimmune diseases have been identified, with some evidence suggesting that there may be more than 100 types. Nearly a ...
, possibly an abortive form of
rheumatoid arthritis Rheumatoid arthritis (RA) is a long-term autoimmune disorder that primarily affects joints. It typically results in warm, swollen, and painful joints. Pain and stiffness often worsen following rest. Most commonly, the wrist and hands are involv ...
.


Presentation

The exact prevalence of palindromic rheumatism in general population is unknown, and this condition is often considered a rare disease by nonrheumatologists. However, recent Canadian study showed that the incidence of PR in a cohort of incident arthritis was one case of PR for every 1.8 cases of rheumatoid arthritis (RA). The incidence of PR is less than that of RA but is not as rare as that was thought to be. Palindromic rheumatism is a syndrome presented with inflammatory para-arthritis (soft tissue rheumatism) and
inflammatory arthritis Inflammatory arthritis is a group of diseases which includes: rheumatoid arthritis, psoriatic arthropathy, inflammatory bowel disease, adult-onset Still's disease, scleroderma, juvenile idiopathic arthritis, and systemic lupus erythematosus (SLE). ...
both of which cause sudden inflammation in one or several joints or soft tissue around joints. The flares usually present with mono- or oligo-articular involvement, which have onset over hours and last a few hours to a few days, and then go away completely. However episodes of recurrence form a pattern, with symptom-free periods between attacks lasting for weeks to months. The most commonly involved joints were knees, metacarpophalangeals and proximal interphalangeals. Constitutionally, there may or may not be a
fever Fever, also referred to as pyrexia, is defined as having a body temperature, temperature above the human body temperature, normal range due to an increase in the body's temperature Human body temperature#Fever, set point. There is not a single ...
, and swelling of the joints. The
soft tissues Soft tissue is all the tissue in the body that is not hardened by the processes of ossification or calcification such as bones and teeth. Soft tissue connects, surrounds or supports internal organs and bones, and includes muscle, tendons, ligam ...
are involved with swelling of the periarticular tissues, especially heel pads and finger pads. Nodules may be found in the
subcutaneous tissues The subcutaneous tissue (), also called the hypodermis, hypoderm (), subcutis, superficial fascia, is the lowermost layer of the integumentary system in vertebrates. The types of cells found in the layer are fibroblasts, adipose cells, and macro ...
. The frequency of attacks may be variable over the course but there is no joint damage after attacks. It typically affects people between the ages of 20 and 50. One study showed an average age of onset of 49. A population cohort study in
Taiwan Taiwan, officially the Republic of China (ROC), is a country in East Asia, at the junction of the East and South China Seas in the northwestern Pacific Ocean, with the People's Republic of China (PRC) to the northwest, Japan to the nort ...
suggested that patients with PR had an increased risk of developing rheumatoid arthritis, systemic lupus erythematosus, Sjogren's syndrome, systemic sclerosis, and polymyositis.


Causes

Palindromic rheumatism is a disease of unknown cause. It has been suggested that it is an abortive form of
rheumatoid arthritis Rheumatoid arthritis (RA) is a long-term autoimmune disorder that primarily affects joints. It typically results in warm, swollen, and painful joints. Pain and stiffness often worsen following rest. Most commonly, the wrist and hands are involv ...
(RA), since anti-
cyclic citrullinated peptide Cycle, cycles, or cyclic may refer to: Anthropology and social sciences * Cyclic history, a theory of history * Cyclical theory, a theory of American political history associated with Arthur Schlesinger, Sr. * Social cycle, various cycles in soc ...
antibodies (anti-CCP) and antikeratin antibodies (AKA) are present in a high proportion of patients, as is the case in
rheumatoid arthritis Rheumatoid arthritis (RA) is a long-term autoimmune disorder that primarily affects joints. It typically results in warm, swollen, and painful joints. Pain and stiffness often worsen following rest. Most commonly, the wrist and hands are involv ...
. Unlike RA and some other forms of arthritis, palindromic rheumatism affects men and women equally. Palindromic rheumatism is frequently the presentation for
Whipple disease Whipple's disease is a rare systemic infectious disease caused by the bacterium '' Tropheryma whipplei''. First described by George Hoyt Whipple in 1907 and commonly considered as a gastrointestinal disorder, Whipple's disease primarily causes m ...
which is caused by the infectious agent ''Tropheryma whipplei'' (formerly ''T. whippelii'').


Diagnosis

Due to the symptoms of palindromic arthritis and the nature of the attacks, diagnosis can be difficult or take a long time. The symptoms can be similar to many other forms of arthritis or other
autoimmune In immunology, autoimmunity is the system of immune responses of an organism against its own healthy cells, tissues and other normal body constituents. Any disease resulting from this type of immune response is termed an "autoimmune disease". P ...
diseases. It is often a case of eliminating the other conditions before getting the correct diagnosis due to there being no specific test for PR diagnosis. No single test can confirm a diagnosis. A
doctor Doctor or The Doctor may refer to: Personal titles * Doctor (title), the holder of an accredited academic degree * A medical practitioner, including: ** Physician ** Surgeon ** Dentist ** Veterinary physician ** Optometrist *Other roles ** ...
may make a diagnosis based on medical history and signs and symptoms. Palindromic rheumatism must be distinguished from acute
gout Gout ( ) is a form of inflammatory arthritis characterized by recurrent attacks of a red, tender, hot and swollen joint, caused by deposition of monosodium urate monohydrate crystals. Pain typically comes on rapidly, reaching maximal intensit ...
y arthritis and an atypical, acute onset of
rheumatoid arthritis Rheumatoid arthritis (RA) is a long-term autoimmune disorder that primarily affects joints. It typically results in warm, swollen, and painful joints. Pain and stiffness often worsen following rest. Most commonly, the wrist and hands are involv ...
(RA). Without specific tests (such as analysis of joint fluid), it may be difficult to distinguish palindromic rheumatism from other episodic joint problems. It is important to note that a person may experience more than one
autoimmune disorder An autoimmune disease is a condition arising from an abnormal immune response to a functioning body part. At least 80 types of autoimmune diseases have been identified, with some evidence suggesting that there may be more than 100 types. Nearly a ...
at the same time, as overlap syndrome.
Laboratory A laboratory (; ; colloquially lab) is a facility that provides controlled conditions in which scientific or technological research, experiments, and measurement may be performed. Laboratory services are provided in a variety of settings: physicia ...
findings are usually normal.
Blood Blood is a body fluid in the circulatory system of humans and other vertebrates that delivers necessary substances such as nutrients and oxygen to the cells, and transports metabolic waste products away from those same cells. Blood in the c ...
tests may show an elevation of the ESR and CRP, but are otherwise unremarkable.
Rheumatoid factor Rheumatoid factor (RF) is the autoantibody that was first found in rheumatoid arthritis. It is defined as an antibody against the Fc portion of IgG and different RFs can recognize different parts of the IgG-Fc. RF and IgG join to form immune com ...
may be present especially in the group that is likely to develop
rheumatoid arthritis Rheumatoid arthritis (RA) is a long-term autoimmune disorder that primarily affects joints. It typically results in warm, swollen, and painful joints. Pain and stiffness often worsen following rest. Most commonly, the wrist and hands are involv ...
. Proposed classification by Guerne and Weismann in 1992: * A 6-month history of brief sudden-onset and recurrent episodes of monoarthritis or rarely polyarthritis or of soft tissue inflammation. * Direct observation of one attack by a physician. * Three or more joints involved in different attacks. * No radiologic evidence of bone or joint erosion. * Exclusion of other arthritides, such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), or gout


Management

Treatment may include nonsteroidal anti-inflammatory drugs (NSAIDs) for acute attacks. Antimalarials, such as hydroxychloroquine, have been helpful in reducing the frequency and duration of attacks and may reduce the likelihood that palindromic rheumatism will progress to rheumatoid arthritis.


Etymology

Palindromic rheumatism derives its name from the Greek meaning to take the same road once again (, again + , pathway) emphasizing how the illness begins and ends in a similar way. The term "
palindrome A palindrome is a word, number, phrase, or other sequence of symbols that reads the same backwards as forwards, such as the words ''madam'' or ''racecar'', the date and time ''11/11/11 11:11,'' and the sentence: "A man, a plan, a canal – Panam ...
" means a word that is spelled the same forward as backward (examples include "kayak" and "mum").


References


External links

{{Diseases of the musculoskeletal system and connective tissue Arthritis Autoimmune diseases Rheumatology Inflammatory polyarthropathies