Periodic/Intermittent Syndromes/Diseases

Wikis > Rheumatology > Periodic/Intermittent Syndromes/Diseases

Periodic/Intermittent Syndromes/Diseases:

Heterogenous group of disorders that have intermittent arthritic symptoms followed by remissions with complete resolution between attacks. Damage to the joint is rare. May be the prodrome of another rheumatic disease.

Intermittent hydrarthrosis:
Rare and benign condition of recurrent joint effusions occurring at regular intervals – usually mono- or pauci-articular. Often follow menses in females. Knee most common. Usually has no symptoms or minimal discomfort or difficulty moving joint. Treatment not usually needed. Some consider it to be a very rare variant of rheumatoid arthritis.

Palindromic Rheumatism:
Recurrent irregular attacks of acute arthritis and periarthritis of short duration. M=F. Usually knee, but 2-5 joints can be involved – can involve small joints of hands and feet. Onset in a few hours (sometimes up to a few days), with a typical pattern each time for each individual. Certain foods, specific for each person, may trigger attack. Pain usually intense with swelling, tenderness and warmth  no residual joint damage. May also get transient subcutaneous nodules. ESR and CRP may be elevated. Swelling of periarticular and soft tissues can occur – especially plantar heel and finger pads. Remission during pregnancy can commonly occur. 30-50% develop a chronic inflammatory arthritis or connective tissue disorder – usually rheumatoid arthritis, especially if have rheumatoid factor and HLA-DR4.
NSAID’s gives symptomatic relief (not necessarily clear if they are helpful due to transient nature of disease process) – intramuscular gold and colchicine have been used; use of antimalarials lessens the risk of developing rheumatoid arthritis .

Familial Mediterranean fever (FMF)/Familial Paroxysmal Polyserisitis/Hereditary Recurrent Polyserositis:
• autosomal recessive disease characterised by a febrile illness and polyserositis of pleura, synovial membranes and the peritoneum. FMF gene on chromosome 16 has been identified (M694V & V726A).
• historically limited to Mediterranean area – now more widespread, especially in those of Mediterranean and Middle eastern origin – especially non-Ashkenazi Jews
• onset usually ages 5-15. Occurs in 1/2600 births in Jordan. Carrier rate of 1/12 Iraqi Jews
• irregular acute onset of fever that is self limited (usually 72hrs). May also get peritonitis (90% have abdominal pain), pleuritis (30%) or arthritis/synovitis (in up to 25% - usually hip, knee or ankle, acute and lasts for less than a week with no residual damage or deformity – often misdiagnosed as a juvenile arthritis). Joint pain and dysfunction are often out of proportion to the swelling – not normally red or warm.
• sometimes get ankle rash (resembles erysipelas) – can feel hot, swollen and tender to touch
• recovery of joint function is usual and damage is rare. Occasionally may get joint damage if attacks are prolonged
• arthritis is presenting symptom in up to 20%
• atypical presentation – case report of initial symptoms as subtalar joint arthritis  later developed fever – treated successfully with oral colchicine
• analgesics help pain; colchicine

Other Periodic Syndromes:
• TNF receptor-associated periodic syndrome (autosomal dominant; caused by mutation in TNFRSF1A gene; characterised by episodic fever, abdominal pain, pleurisy, arthralgia and a kin rash; treated with colchicine)
• Muckle-Wells syndrome (episodic inflammation, sensorineural deafness beginning in childhood)
• Eosinophilic synovitis (acute self-limiting episodes of monoarthritis triggered by trauma with history of allergy)

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