| Reiter's Syndrome | |
|---|---|
| Classification and external resources | |
Diagnosis revealed that the rash on the bottom of this individual’s feet, known as keratoderma blennorrhagica, was due to Reiter's syndrome. |
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| ICD-10 | M02 |
| ICD-9 | 099.3 |
| DiseasesDB | 29524 |
| eMedicine | med/1998 |
| MeSH | C01.539.100.500 |
Reactive arthritis (Reiter's Syndrome or Reiter's arthritis), is classified as an autoimmune condition that develops in response to an infection in another part of the body (cross-reactivity). Coming into contact with bacteria and developing an infection can trigger the disease.[1] Reiter's syndrome has symptoms similar to various other conditions collectively known as "arthritis". By the time the patient presents with symptoms, often time the "trigger" infection has been cured or is in remission in chronic cases, thus making determination of the initial cause difficult.
Commonly known as Reiter's syndrome, recent political pressure has seen certain institutions utilize "reactive arthritis" but the condition is also known as arthritis urethritica, venereal arthritis and polyarteritis enterica. It is a type of seronegative spondyloarthropathy. The original name "Reiter’s syndrome", named after the German physician Hans Conrad Julius Reiter for his contributions to identification and description of the disease, has become unpopular in the past decade as Reiter's history of Nazi party membership, forced human experimentation in the Buchenwald concentration camp, and subsequent prosecution in Nuremburg as a war criminal, have come to light.[2]
The manifestations of Reactive arthritis include the following triad of symptoms: an inflammatory arthritis of large joints including commonly the knee and the back (due to involvement of the sacroiliac joint), inflammation of the eyes in the form of conjunctivitis or uveitis, and urethritis in men or cervicitis in women. Patients can also present with mucocutaneous lesions, as well as psoriasis-like skin lesions such as circinate balanitis, and keratoderma blennorrhagica. Enthesitis can involve the Achilles tendon resulting in heel pain.[3] Not all affected persons have all the manifestations, and the formal definition of the disease is the occurrence of otherwise unexplained non-infectious inflammatory arthritis combined with urethritis in men, or cervicitis in women.
Reiter's syndrome is an RF-seronegative, HLA-B27-linked spondyloarthropathy[4] (autoimmune damage to the cartilages of joints) often precipitated by genitourinary or gastrointestinal infections. The most common triggers are sexually transmitted Chlamydial infections and perhaps, less commonly, Neisseria gonorrhea; and Salmonella, Shigella, or Campylobacter intestinal infections. There is evidence to suggest that BCG vaccine used for bladder instillation in treating bladder CIS tumour can trigger Reactive Arthritis. Symptoms can start from a week to six weeks after the last instillation and further instillations can exacerbate the condition.
Reactive arthritis most commonly strikes individuals aged 20–40 years of age, and is more common in men than in women, and more common in whites than in blacks. This is owing to the high frequency the of HLA-B27 gene in the white population.[5][6] Patients with HIV have an increased risk of developing reactive arthritis as well.
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Symptoms generally appear within 1–3 weeks but can range from 4 to 35 days from the onset of the inciting episode of the disease.
The classical presentation is that the first symptom experienced is a urinary symptom such as burning pain on urination (dysuria) or an increased frequency of urination. Other urogenital problems may arise such as prostatitis in men and cervicitis, salpingitis and/or vulvovaginitis in women. The arthritis that follows usually affects the large joints such as the knees causing pain and swelling with relative sparing of small joints such as the wrist and hand.
Eye involvement occurs in about 50% of men with urogenital reactive arthritis and about 75% of men with enteric reactive arthritis. Conjunctivitis and uveitis can include redness of the eyes, eye pain and irritation, or blurred vision. Eye involvement typically occurs early in the course of reactive arthritis, and symptoms may come and go.
Roughly 20 to 40 percent of the men with the disease develop penile lesions called balanitis circinata (circinate balanitis). A small percentage of men and women develop small hard nodules called keratoderma blennorrhagica on the soles of the feet and, less commonly, on the palms of the hands or elsewhere. In addition, some individuals with reactive arthritis develop mouth ulcers that come and go. In some cases, these ulcers are painless and go unnoticed. Some patients suffer serious gastrointestinal problems similar to those of the Crohn's disease.
About 10 percent of the people with reactive arthritis, especially those with a prolonged course of the disease, will develop cardiac manifestations, including aortic regurgitation and pericarditis. Reiter's Syndrome has been described as a pre-cursor to other seronegative spondylarthropathies, including ankylosing spondylitis.
A common mnemonic for the syndrome is "Can't see, can't pee, can't climb a tree." Although useful, critics of the mnemonic have pointed out that sufferers can in fact urinate, although it is painful.
In the oral cavity, the patients may suffer from recurrent aphthous stomatitis, geographic tongue and migratory stomatitis in higher prevalence than the general population.[7]
It is set off by a preceding infection, the most common of which would be a genital infection with Chlamydia trachomatis in the US. Other bacteria known to cause reactive arthritis which are more common worldwide are Ureaplasma urealyticum, Salmonella spp., Shigella spp., Yersinia spp., and Campylobacter spp.[8] A bout of food poisoning or a gastrointestinal infection may also precede the disease (those last four genera of bacteria mentioned are enteric bacteria). There is some circumstantial evidence for other organisms causing the disease, but the details are unclear.[9] Reactive arthritis usually manifests about 1–3 weeks after a known infection. The mechanism of interaction between the infecting organism and the host is unknown. Synovial fluid cultures are negative, suggesting that reactive arthritis is caused either by an over-stimulated autoimmune response or by bacterial antigens which have somehow become deposited in the joints.
There are few clinical symptoms, but the clinical picture is dominated by polyarthritis. There is pain, swelling, redness, and heat in the joints affected. MRI is effective in diagnosis.
The urethra, cervix and the throat may be swabbed in an attempt to culture the causative organisms. Cultures may be carried out on urine and stool samples. Arthrocentesis can be done in order to study the synovial fluid from an affected joint for further cell count, and for culture.
Also, a blood test for the genetic marker HLA-B27 may be given to determine if the patient has the gene. About 75 percent of all the patients with Reiter's arthritis have the gene. C-Reactive Protein (CRP), and Erythrocyte Sedimentation Rate (ESR) are non-specific tests that can be done to corroborate the diagnosis of the syndrome.
Although there are no definitive criteria to diagnose the existence of Reiter's arthritis, the American College of Rheumatology has published sensitivity and specificity guidelines.[10]
| Percent Sensitivity and Specificity of Various Criteria for Typical Reiter's Syndrome | ||
| Method of diagnosis | Sensitivity | Specificity |
| 1. Episode of arthritis of more than 1 month with urethritis and/or cervicitis | 84.3% | 98.2% |
| 2. Episode of arthritis of more than 1 month and either urethritis or cervicitis, or bilateral conjunctivitis | 85.5% | 96.4% |
| 3. Episode of arthritis, conjunctivitis, and urethritis | 50.6% | 98.8% |
| 4. Episode of arthritis of more than 1 month, conjunctivitis, and urethritis | 48.2% | 98.8% |
The main goal of treatment is to identify and eradicate the underlying infectious source with the appropriate antibiotics if still present. Otherwise, treatment is symptomatic for each problem. Analgesics particularly NSAIDs, steroids and immunosuppressants may be needed for patients with severe reactive symptoms that do not respond to any other treatment.
Reactive arthritis may be self-limiting, frequently recurring, chronic or progressive. Most patients have severe symptoms lasting a few weeks to six months. Fifteen to 50 percent of cases have recurrent bouts of arthritis. Chronic arthritis or sacroiliitis occurs in 15-30 percent of cases. Repeated attacks over many years are common, and patients sometimes end up with chronic and disabling arthritis, heart disease, amyloid deposits, ankylosing spondylitis, immunoglobulin A nephropathy, cardiac conduction abnormalities, or aortitis with aortic regurgitation.[11] However, most people with reactive arthritis can expect to live normal life spans and maintain a near-normal lifestyle with modest adaptations to protect the involved organs.
Because women may be underdiagnosed, the exact incidence of reactive arthritis is difficult to estimate. A few studies have been completed, though. In Norway between 1988 and 1990, incidence was 4.6 cases per 100,000 for Chlamydia-induced reactive arthritis and 5 cases per 100,000 for that induced by enteric bacteria.[12] In 1978 in Finland, the annual incidence was found to be 43.6 per 100,000.[13]
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 France), and Reiter documented the case of a single patient with this triad of symptoms (in Germany). In 1942, an article by Bauer and Engelman described the first known American patient with reactive arthritis; 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 in use despite its historical inappropriateness and Hans Reiter's later activities as a National Socialist (Nazi) war criminal.
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