Chronic Post-Rheumatic Polyarthrosis: What is Jaccoud's Arthropathy?
Over time our understanding of diseases deepens and evolves, for the scientific community as well as the patient community. This is certainly true for rheumatic and arthritic diseases. Today, I'd like to introduce you to one type of arthritis which is not widely discussed in the patient community.
What is Jaccoud's arthropathy?
In 1869, F.S. Jaccoud, a French clinician, described cases of arthritis in patients following frequent and severe attacks of rheumatic fever. This type of arthritis was also known as chronic post-rheumatic polyarthrosis. Joint deformities are primarily in the hands, characterized by muscle atrophy, severe ulnar deviation and flexion at the big knuckle (or metacarpal-phalangeal) joints. It resembles rheumatoid arthritis, but with less inflammation and serum tests negative for Rheumatoid Factor.
Since -itis refers to inflammation, and since Jaccoud's does not involve chronic inflammation as does rheumatoid arthritis, chronic post-rheumatic polyarthrosis is more commonly referred to as Jaccoud's arthropathy. But you will find both names used interchangeably in the research literature.
What is Rheumatic Fever?
Rheumatic fever is an inflammatory disease affecting connective tissues throughout the body, specifically involving the heart, joints, brain, and skin. It can develop after infection with the streptococcus bacteria which causes strep throat and scarlet fever. Rheumatic fever is no longer common in the United States, but it is the leading cause of rheumatic heart disease throughout the world.
According to the American Heart Association, symptoms of rheumatic fever include: fever; painful, tender, red swollen joints; pain in one joint that migrates to another one; heart palpitations; chest pain; shortness of breath; skin rashes; fatigue; and small, painless nodules under the skin. The symptoms to of rheumatic fever usually appear about three weeks after strep throat or scarlet fever. The best prevention for rheumatic fever is to treat the streptococcus infection with antibiotics.
What causes Jaccoud's arthritis?
Rheumatic fever can be one trigger of Jaccoud's arthropathy, however the exact cause is not clear. In the 1950s, E.G. Bywaters pointed out the similarities between the arthritis reported by Jaccoud and joint deformities observed in patients with systemic lupus erythematosis (SLE), also known as lupus. In conducting research for this article, I found several sources discussing Jaccoud's arthropathy in association with rheumatic fever and lupus (SLE). (see Musculoskeletal Manifestations of Systemic Lupus Erythematosus) However, I also found several isolated case reports such as one published in Rheumatology International (2010, 30(8):1081-3), in which Jaccoud's arthropathy was seen in patients with long-standing psoriatic arthritis. Again, having one arthritic disease does not preclude developing another.
Jaccoud's arthropathy has been reported in cases of rheumatic fever, lupus (SLE), scleroderma (systemic sclerosis), psoriatic arthritis, dermatomyositis, urticarial hypocomplementic vasculitis, systemic necrotizing vasculitis, malignancies, and ulcerative colitis. Isolated cases have been reported in nonautoimmune rheumatic diseases such as keratitis ichthyosis deafness (KID) syndrome, AIDS, mycosis fungoides, angioimmunoblastic lymphadenopathy and Calort syndrome.
Does Jaccoud's arthropathy cause permanent damage?
Unlike the permanent damage seen in rheumatoid arthritis, the deformities caused by Jaccoud's arthropathy (flexion deformities, ulnar deviation, soft tissue laxity, and swan neck deformities) can be corrected either voluntarily (under the patients own power) or passively. In Jaccoud's arthropathy, loose ligaments, tendon fibrosis, and muscle imbalance, rather than the loss of bone and joint instability secondary to synovitis, cause the deformities which more commonly involve the hands, but can also affect the wrists, feet, ankles, and knees. (see Ann Rheum Dis 2008;67:728-729)
It was previously thought that Jaccoud's arthritis did not cause bone erosions, however we now know that it does. Bone erosions may not be seen on x-rays, however erosions can be detected by Ultrasound examination. (see Journal of Clinical Rheumatology Dec 2007;13(6):322-327) Erosions are more commonly seen at the end of the metacarpal heads.
Jaccoud's arthropathy is related more to inflammation of the tendon sheath than the synovium. In a study of 55 patients diagnosed with lupus (SLE) who experienced spontaneous tendon ruptures (most commonly in the patellar and Achilles' tendons), it was found that one-third (16 patients) also had Jaccoud's arthropathy. Researchers suggest that Jaccoud's arthropathy should be recognized as a risk marker for tendon rupture. (see Lupus 2010;19:247-254)
How is Jaccoud's arthropathy diagnosed?
E.G. Bywaters summarized the characteristics of Jaccoud's arthritis as foliows: (see Calif Med 1970 April;112(4):19-22)
***** There is a history of severe rheumatic fever with repetitive or prolonged attacks, associated with heart disease, chorea and migratory polyarthritis.
***** Recovery may be delayed and it is associated with stiffness in the metacarpal joints, which may clear or may cause deformities.
***** The characteristic deformity appears to be periarticular, fascial and tendon fibrosis rather than synovitis.
***** Deformity is due to flexion at the metacarpal phalangeal joints, associated with periarticular soft tissue swelling and ulnar deviation.
***** Joint disease is inactive, with few or no symptoms, good functional capacity, normal sedimentation rate and a negative test for rheumatoid factor.
***** X-ray studies rarely show the typical changes associated with rheumatoid arthritis.