Excerpt from Reiter Syndrome

Synonyms, Key Words, and Related Terms: RS, Reiter disease, Fiessinger-Leroy-Reiter syndrome, Fiessinger-Leroy syndrome, arthritis urethritica, blennorrheal idiopathic arthritis, reactive arthritis, conjunctivo-urethro-synovial syndrome, polyarthritis enterica, keratoderma blenorrhagica, urethritis

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Background: Reiter syndrome (RS) is a systemic disorder of unknown etiology that is defined by the development of psoriatic plaques, balanitis, keratoderma, conjunctivitis, urethritis, arthritis, and spondylitis. This symptom complex usually follows an episode of either dysentery or urethritis.

The American Rheumatism Association criteria subcommittee defined this syndrome as 1 month of peripheral arthritis associated with urethritis, cervicitis, or both. The classic triad of the disease, namely urethritis, arthritis, and conjunctivitis, is present in only one third of the patients.

Stoll originally described this triad in 1776. In 1818, Brodie reported the triad in 5 patients. In 1916, 2 separate reports were published during World War I: Fiessinger and Leroy detailed the findings in 4 patients (in French), and Reiter documented the case of a single patient with this triad of symptoms (in German). In 1942, an article by Bauer and Engelman described the first known American patient with RS; they called this disorder, a "syndrome of unknown etiology characterized by urethritis, conjunctivitis, and arthritis (so-called Reiter's disease)." Their work contained only one reference, Reiter's article, and stated erroneously, "First described by Reiter, it has been most commonly referred to as Reiter's disease." Thus, this eponym remains despite its historical inappropriateness and Hans Reiter's later activities as a National Socialist war criminal.

RS mostly affects young men. It is frequently associated with the human leukocyte antigen B27 (HLA-B27) haplotype and is classified with the seronegative spondyloarthropathies. RS is preferably viewed as a tetrad, with the addition of the mucocutaneous findings of balanitis and keratoderma blennorrhagicum to the classic triad. The complete and incomplete forms of RS can be identified by the presence or absence of the full tetrad.

Young children tend to have the postdysenteric form, whereas adolescents and young men are most likely to acquire RS after they have urethritis. This association with preceding infection has lead to RS being called a reactive arthritis. Interpreting its mucocutaneous findings as pustular psoriasis and its seronegative arthritis as psoriatic arthritis, some believe that RS is best classified as a type of psoriasis.

Pathophysiology: The etiology of Reiter disease remains uncertain. Two forms are recognized: a sexually transmitted form and a dysenteric form. Because the urethritis is a possible primary event, research efforts have focused on the identification of a microorganism that could be responsible for activating this disease. The pathophysiologic mechanism is proposed to be the triggering of an autoimmune reaction by these microorganisms.

Mycoplasma (Ureaplasma) species, Neisseria gonorrhoeae, Chlamydia species, and several viruses are among the suspected causative pathogens. Some findings have indicated that Chlamydia species are the etiologic agents in RS. The recent discovery of Chlamydia trachomatis organisms in an involved joint and the confirmation of an immune response against Chlamydia infection (as indicated by high titers of antichlamydial antibodies in serum) have provided additional support to this hypothesis. In situ hybridization has also been used to identify chlamydial infection in synovial tissue. Ureaplasma organisms can cause experimental and clinical nongonococcal urethritis. Synovial mononuclear cells from arthritic joints of patients with RS react with Ureaplasma antigens; this organism has been isolated from a patient.

RS is also reported to occur after enteric bacterial infections, primarily those caused by parasites (Ascaris lumbricoides) and Shigella, Salmonella, Yersinia, Clostridium, and Campylobacter organisms. Recently, impairment in the glycosaminoglyc .....