Reiter's Disease & Reactive arthritis
*Synonyms and related keywords:
Reiter's syndrome, RS, reactive arthritis, ReA, nongonococcal urethritis, conjunctivitis, oculo-urethro-synovial syndrome, Shigella dysentery, gastrointestinal infections, Salmonella, Campylobacter, Chlamydia trachomatis, C trachomatis, Yersinia, ankylosing spondylitis, psoriatic arthritis, seronegative spondyloarthropathy, infectious diarrhea, genitourinary infection*
Background:
In 1916, Hans Reiter described a triad of nongonococcal urethritis, conjunctivitis, and arthritis in a young German officer who had a bout of bloody dysentery. In 1916, Fiessinger and Leroy described 4 patients with what they called oculo-urethro-synovial syndrome and associated the syndrome with an outbreak of Shigella dysentery.*
Pathophysiology:
Reactive arthritis usually develops 2-4 weeks after a genitourinary or gastrointestinal infection. Recent evidence indicates that a preceding respiratory infection with Chlamydia may also trigger the disease. About 10% of patients do not have a preceding symptomatic infection.*
Inflammation of joints, entheses, axial skeleton, skin, mucous membranes, gastrointestinal tract, and eyes may occur. HLA-B27 is positive in 65-96% of patients (75% on average). Patients who are HLA-B27 positive have about a 50-fold increased chance of developing reactive arthritis, but this syndrome can occur in patients who are HLA-B27 negative.
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Race: Prevalence of HLA-B27 and reactive arthritis is higher in white people than in black people, as in other spondyloarthropathies. Sex: Reactive arthritis following food-borne enteric infections affects males and females with the same frequency. Disease associated with venereally acquired infections occurs in a male-to-female ratio of 9:1. Age: Most patients are aged 20-40 years.
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History:
Reactive arthritis usually develops 2-4 weeks after a genitourinary or gastrointestinal infection. Recent evidence indicates that a preceding respiratory infection with Chlamydia pneumoniae may also trigger the disease. About 10% of patients do not have a preceding symptomatic infection. Nongonococcal urethritis, if present, can be one of the presenting symptoms for both postvenereal and postenteric forms. Mild dysuria, mucopurulent discharge, prostatitis and epididymitis in men, and vaginal discharge and/or cervicitis in women can be observed. The onset is most often acute, with malaise, fatigue, and fever. An asymmetrical predominately lower extremity oligoarthritis is the major presenting symptom. Low back pain occurs in 50% of patients. Heel pain is common because of enthesopathies at the Achilles or plantar aponeurosis insertion on the calcaneus. The complete Reiter triad of urethritis, conjunctivitis, and arthritis may occur.
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Physical: Joints, axial skeleton, entheses Peripheral joint involvement is typically asymmetric and most frequently affects the weight-bearing joints (ie, knees, ankles, hips), but the shoulders, wrists, and elbows are also affected. In more chronic and severe cases, the small joints of the hands and feet can also be involved. Dactylitis (ie, sausage digits) can be observed, as in other spondyloarthropathies. While low back pain may be present in 50% of patients, most patients with acute disease have minimal findings on physical examination except for decreased lumbar flexion. Patients with more chronic and severe axial disease may develop physical findings similar to ankylosing spondylitis. As with other spondyloarthropathies, the enthesopathy of reactive arthritis may be associated with findings of inflammation (ie, pain, tenderness, swelling) at the Achilles insertion. Other sites include the plantar fascial insertion on the calcaneus, ischial tuberosities, iliac crests, tibial tuberosities, and ribs.
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Skin and nails Keratoderma blennorrhagica observed in the palms and soles is indistinguishable from pustular psoriasis and is very suggestive of chronic reactive arthritis. Erythema nodosum can be observed but is uncommon. Nails can become thickened and crumble, resembling mycotic infection or psoriatic onychodystrophy, but nail pitting is not observed. Circinate balanitis can also be observed. Other mucosal signs and symptoms: Painless shiny patches in the palate, tongue, and mucosa of the cheeks and lips have been described.
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Ocular findings Conjunctivitis is part of the classic triad of Reiter syndrome, and it can occur before or at the onset of arthritis. Other ocular lesions include acute uveitis (20% of patients), episcleritis, keratitis, and corneal ulcerations. The lesions have a tendency to recur. Enteric infections Enteric infections can be the triggering event for reactive arthritis. Pathogens include Salmonella, Shigella, Yersinia, and Campylobacter species. The frequency of reactive arthritis after these enteric infections is about 1-4%. Some patients continue with intermittent bouts of diarrhea and abdominal pain. Lesions resembling ulcerative colitis or Crohn disease have been described when ileocolonoscopy is performed in patients with established reactive arthritis. Other manifestations Other manifestations of the disease include mild renal pathology with proteinuria and microhematuria. In severe chronic cases, amyloid deposits and immunoglobulin A (IgA) nephropathy have been reported. Cardiac conduction abnormalities can be observed, and aortitis with aortic regurgitation occurs in 1-2% of patients. Causes: Reactive arthritis is usually triggered by a genitourinary or gastrointestinal infection.
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Lab Studies
Acute phase reactants, including (ESR) and (CRP), are usually elevated markedly but later return to the reference range with subsidence of the inflammation. normocytic normochromic anemia along with mild leukocytosis and thrombocytosis during the acute phase. IgA antibodies to specific bacterial antigens have been reported. Urinalysis can show aseptic pyuria. Synovial fluid analysis reveals a high white blood cell count, Gram stain and culture results are negative and are necessary to exclude septic arthritis. Throat, stool, or urogenital tract cultures can be performed in an attempt to isolate the causative organism. Test results for rheumatoid factor and antinuclear antibodies are negative.*
Imaging Studies:
Early in the disease, no abnormalities are found on radiograph. In more advanced or long-term disease, periosteal reaction and proliferation at sites of tendon insertion can be observed. Exuberant plantar spurs are a common sign in long-term cases. In the hands and feet, marginal erosions with adjacent bone proliferation occur. Spinal radiographic findings include sacroiliitis and syndesmophytes. Sacroiliitis occurs in less than 10% of acute cases but may be observed in 50% of those patients with chronic severe disease. Syndesmophytes are usually asymmetrical and are found most commonly in the thoracolumbar region. Severe ankylosing spondylitis occurs in less than 5% of cases.*
Treatment
The treatment of reactive arthritis is modified according to the severity of symptoms. Physical therapy may be instituted to avoid muscle wasting and to reduce pain. Nonsteroidal anti-inflammatory drugs Corticosteroids Antibiotics Disease-modifying antirheumatic drugs*
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Pathophysiology:
Psoriatic arthritis is an autoimmune disease with known human leukocyte antigen (HLA)–associated risk factors. Psoriatic arthritis affects the ligaments, tendons, fascia, and joints, and it occasionally develops in the absence of detectable psoriasis. *Race: Psoriatic arthritis is more common in white persons than in persons of other races. Sex: Men and women are affected equally; however, if the subsets of psoriatic arthritis are considered, male predominance occurs in the spondylitic form, whereas female predominance occurs in the rheumatoid form. Age: Psoriatic arthritis characteristically develops in persons aged 35-55 years, but it can occur in persons of almost any age.
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History:
Psoriatic arthritis may be present with or without obvious skin lesions, with minimal skin involvement (eg, scalp, umbilicus, intergluteal cleft), or with only nail malformations. Psoriasis usually precedes arthritis (occasionally by as many as 20 y); however, in as many as 15-20% of patients, arthritis appears before the psoriasis. If the latter is the case, a family history of psoriasis may reveal a hereditary pattern.*
The following list details the 5 patterns of psoriatic arthritis involvement:
Asymmetrical oligoarticular arthritis (the typical "sausage" appearance (dactylitis). Symmetrical polyarthritis DIP arthropathy Arthritis mutilans Spondylitis with or without sacroiliitis Juvenile psoriatic arthritis*
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The pencil-in-cup deformity observed in the hands and feet of patients
*Behcet's disease
*Synonyms and related keywords:
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Background:
*Sex: Men are affected more often, and with more severe disease, than women in some Mediterranean areas. In Iran, for example, the male-to-female ratio was 24:1 among 1712 patients. In Turkey, the ratio was 16:1 among 427 patients. Age: Onset can occur at any age, but is it most common during the third decade of life.
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This complex, multisystemic disease includes involvement of the mucocutaneous, ocular, cardiovascular, renal, gastrointestinal, pulmonary, urologic, and central nervous systems and the joints, blood vessels, and lungs.
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History:
Signs and symptoms, which may be recurrent, may precede the onset of the mucosal membrane ulcerations by 6 months to 5 years. Prior to the onset of BD, patients may experience a variety of symptoms. Malaise Anorexia Weight loss Generalized weakness Headache Perspiration Decreased or elevated temperature Lymphadenopathy Pain of the substernal and temporal regions*
Diagnostic criteria from the Behзet syndrome research committee of Japan (1987 revision) are as follows:Major features Recurrent aphthous ulceration of the oral mucous membrane Skin lesions - Erythema nodosum–like lesions, subcutaneous thrombophlebitis, folliculitis (acnelike lesions), cutaneous hypersensitivity Eye lesions - Iridocyclitis, chorioretinitis, retinouveitis, definite history of chorioretinitis or retinouveitis Genital ulcersMinor features Arthritis without deformity and ankylosis Gastrointestinal lesions characterized by ileocecal ulcers Epididymitis Vascular lesions Central nervous system symptoms *
Positive pathergy test - An erythematous papule larger than 2 mm at the prick site 48 hours after the application of a 20- to 22-gauge sterile needle, which obliquely penetrated avascular skin to a depth of 5 mm as read by a physician at 48 hours
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Treatment for Behcet's disease
Currently there is no cure for Behcet's disease. The main goal is to treat and manage the symptoms so that complications do not develop. Because Behcet's disease affects so many different parts of the body, a team of doctors from various specialties will treat most people.*
Topical therapy Tetracycline solution Topical corticosteroids Local anaesthetics Systemic therapy Oral corticosteroids Nonsteroidal anti-inflammatory drugs (e.g. aspirin, ibuprofen) Immunosuppressants (e.g. azathioprine, chlorambucil) Experimental and research drugs (e.g. cyclophosphamide, thalidomide)
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Whipple Disease
Synonyms and related keywords: Whipple's disease, Tropheryma whippelii,*
Background: Whipple disease is a systemic disease most likely caused by a gram-positive bacterium, Tropheryma whippelii.
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Race: Whipple disease is most common in white males and rarely is described in females. Sex: Males predominate, roughly 8-9:1. Age: Whipple disease is usually observed in middle-aged and elderly persons (older than 40 y).
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History:
The classic presentation is that of a wasting illness characterized by arthralgias, arthritis, fever, and diarrhea. Lymphadenopathy may be present. If the disease affects the small intestine, steatorrhea often is present. Approximately 90% of patients present with weight loss, and 70% of patients complain of either diarrhea or arthralgias. Occult GI bleeding can be found in 80% of patients, but frank hematochezia is uncommon. Cardiac involvement occurs in approximately 30% of cases.*
Physical:
*The mainstay of medical treatment is antibiotic therapy.
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