Jaccoud’s arthritis or chronic postrheumatic-fever arthritis is a syndrome which associates a chronic relatively asymptomatic severe joint deformity with preceding episodes of endocarditis. Its etiology and pathophysiology remain unclear. Although the term arthritis implies an inflammatory process, cardinal signs of inflammation are not a part of this syndrome. Its hallmark is severe deformity without joint destruction. The term Jaccoud’s arthritis is a misnomer and should be replaced by the term Jaccoud’s arthropathy.
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| Jaccoud’s arthritis |
Through the years, there has been an interest in the relationship of rheumatic fever, valvular heart disease and rheumatoid arthritis. There appears to be a spectrum between pure rheumatic heart disease and pure rheumatoid arthritis.
📖 Firestein & Kelley’s Textbook of Rheumatology
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| Jaccoud arthropathy on the hands, with ulnar deviation of the MCP, ‘gooseneck’ deformity and ‘Z’ thumb deviation with these deformities being more pronounced on the right hand |
Occasionally, the 2 classic patterns will present in the same patient. However, when joint involvement occurs with valvular heart disease, it is not always possible to discern the etiologic relationship. It seems clear that the process which produces rheumatoid arthritis may also produce rheumatoid heart disease with characteristic rheumatoid nodules in both the synovium and myocardium. The other fairly clear relationship is the association of rheumatic fever with a relatively asymptomatic, chronic arthropathy, called Jaccoud’s or postrheumatic-fever arthritis.
In 1867, S. Jaccoud first described the clinical syndrome which bears his name in a 29 year old man with recurrent polyarthritis and rheumatic fever. After several bouts of polyarthritis of the large joints, each lasting several weeks, the patient developed endocarditis with murmurs of aortic stenosis and insufficiency as well as symptoms of congestive heart failure. In mid course, the polyarthritis moved from the large joints to the small joints of the hands and feet. During the fifth episode of polyarthritis, which lasted 5 months, deformities of the hands and feet began to appear. After the sixth bout of arthritis, also lasting 5 months, the patient was left with a severe arthropathy which initially was passively reducible, but later became permanently fixed. When the patient was presented at a lecture about 8 months after the last attack, his hands showed muscle atrophy, severe ulnar deviation with flexion and subluxation at the metacarpophalangeal joints, and hyperextension of the distal phalanges. The thumbs and wrists were not involved, but the toes and elbows were. Jaccoud categorized this as a case of chronic rheumatism following acute rheumatic fever.
Bywaters reviewed the world literature concerning the relationship of heart and joint disease. He mentioned several cases reported between 1881 and 1935 that fit the category of chronic rheumatism following acute rheumatic fever and then added 2 cases of his own. Subsequently, 9 cases were reported in the English language literature.
Because Jaccoud’s arthropathy is usually asymptomatic, most cases of this syndrome are described at the end stage. This circumstance limits meaningful interpretation and elucidation of pathogenesis. The few biopsy specimens obtained indicate that the arthropathy is characterized by normal synovium and articular surfaces with periarticular (capsular) fibrosis.From this information, no accurate statement about the natural pathophysiologic development of the arthropathy can be evolved. It cannot be said with any certainty that the microscopic periarticular fibrosis demonstrated in the end stage arthropathy has any responsibility for that arthropathy.
Sigismond Jaccoud (1830 - 1913), Swiss physician.
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| Sigismond Jaccoud |
Sigismond Jaccoud was born in 1830 in Geneva, where he went to school and was educated in music and the science of literature. In 1849 he went to Paris to study medicine - and supported himself in that city teaching music and literature. He became interne des hôpitaux in 1855. After graduation in 1859 he specialised in internal medicine and in 1860 defended his doctoral thesis, on the pathogenesis of albuminuria. In 1862 he became médecin des hôpitaux, in 1863 professeur agrégé. In 1877 he was appointed professor of internal pathology at the medical faculty and member of the Académie des Médecins.
Jaccoud was a very famous and highly estimated lecturer at several of Paris' hospitals - L'Hôpital Saint-Antonie, l'Hôpital de la Charité, l`Hôpital de la Lariboisière and l'Hôpital de la Pitié. Following the death of Ernest-Charles Lasègue (1816-1883) in 1883, he was also made professor of internal medicine at the Pitié hospital in Paris.
A Swiss, Jaccoud was a very popular lecturer in Paris' hospitals in the late 1800's. In 1883 he published a three-volume work on pathology, comprising almost 3.000 pages. In rheumatology, and partly in cardiology, Jaccoud was probably best known for his 23. Lecture, which has been perpetuated in medical history because of its description of Jaccoud's syndrome. At the turn of the century rheumatic fever ravaged among children and youth, and the fact that there was, unlike today, no rational pharmaceutical therapy available, interest concentrated on the natural course of the disease.
As he published his lectures in book form, they are still available for study - covering an impressive variety of medical questions. On tuberculosis, the greatest medical problem of the time, with its numerous complications, no less than ten lectures were needed.
In his books he emphasises how he enjoys thorough clinical examinations, epidemiology, research and teaching.
Jaccoud died i 1913, at the age of 83 years.
References
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