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Hanna Przepiera-Będzak Klinika Reumatologii PAM, Szczecin. THE SPONDYLOARTHROPATHIES encompass a family of chronic inflammatory disorders primarily affecting peripheral and axial joints. Unifying features include:
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Hanna Przepiera-Będzak Klinika Reumatologii PAM, Szczecin
THE SPONDYLOARTHROPATHIES encompass a family of chronic inflammatory disorders primarily affecting peripheral and axial joints. Unifying features include: • A tendency for involvement of sacroiliac and other spinal joints (sacroiliitis and spondylitis) • Peripheral arthritis, typically asymmetrical and oligoarticular • Inflammatory lesions of tendon and fascial insertions (enthesopathy) at both peripheral and axial sites • Similar extra-articular complications affecting the eye (anterior uveitis) and/or heart (aortitis and conduction disturbance) • Disease onset in young adults, especially men • The absence of rheumatoid factor and other autoantibodies • Strong associations with class I histocompatibility antigens, especiallyHLA-B27
MEMBERS OF THE SPONDYLOARTHROPATHY FAMILY INCLUDE ANKYLOSING SPONDYLITIS, a predominantly axial arthritis usually beginning in the sacroiliac joints and slowly progressing to spinal fusion REITER'S SYNDROME or reactive arthritis, an acute peripheral arthritis associated with nongonococcal urethritis, conjunctivitis, and/or other mucocutaneous features that typically follows enteric or genitourinary infections PSORIATIC ARTHRITIS, a slowly progressive peripheral arthritis, with or without axial disease, occurring in the setting of cutaneous psoriasis ENTEROPATHIC ARTHRITIS, a peripheral and/or axial arthropathy accompanying the idiopathic inflammatory bowel
PREVALENCE AND EPIDEMIOLOGY ANKYLOSING SPONDYLITIS 0.1% to 0.2% of American and European Caucasians and it affects men more often than women (ratio 3:1) REITER'S SYNDROME the prevalence of in most populations is unknown PSORIATIC ARTHRITIS develops in only 5% to 7% of persons with psoriasis prevalence of cutaneous psoriasis 2% of Caucasians). Males and females are equally affected. PERIPHERAL ARTHRITIS occurs in only 20% and Spondylitis in 10% of patients with ulcerative colitis and Crohn's disease
THE SERONEGATIVE SPONDYLOARTHROPATHIESCOMMON DISEASE MANIFESTATION • seronegativity for rheumatoid factors • absence of antinuclear antibodies • absence of rheumatic nodules • peripheral arthritis • genitourinary (urethritis, vaginitis, balanitis) • mucocutaneous (stomatitis, dermatitis • Intestinal (enteric infection or chronic inflammation) • radiological sacroilitis • genetic (familial aggregation, HLA-B27)
CHARACTERISTICS OF INFLAMMATORY BACK PAIN - onset of pain before the age of 40 - more or less persistent for at least 3 months - gradual onset - early morning stiffness lasting more than 15 minutes - improvement on movement If four or more of these features are present, the back pain is likely to be of inflammatory origin.
ANKYLOSING SPONDYLITIS Patients usually describe a gradual onset of low back pain over 3 or more months. The pain is described as follows: • Worse in the morning with improvement during the day • Better with activity and worse with rest (helps in distinguishing AS from mechanical low back pain) Gradual ascending pattern from the lumbar region to the thoracic and then the cervical spine Approximately 25% of patients present with complaints of proximal joint involvement. Rarely, small joint involvement is a presenting feature. Patients may describe pain and stiffness of the rib cage, which may or may not be pleuritic in nature. Atypical chest pain may be present.
ANKYLOSING SPONDYLITIS Causes: • About 90-95% of patients have the HLA-B27 antigen. • Onset and flare-ups may be due to poorly understood environmental factors. • Presumably, a fairly benign bacterium or virus can be antigenically similar to human ligaments. • In a susceptible individual, a mild infection might stimulate an abnormal immune response.
ANKYLOSING SPONDYLITIS – CLINICAL SYMPTOMS Any patient under 40 years who for at least three months has had low back pain which is worse at rest - in particular late at night or in the early morning - may have sacroiliitis. If the symptoms are of gradual onset and include morning stiffness that lasts more than 15 minutes and tends to decrease with movement, the condition is likely to be of inflammatory origin. The pain typically radiates to the buttock and along the upper leg to the knee. ON PHYSICAL EXAMINATION THE FOLLOWING FEATURES ARE COMPATIBLE WITH SERO-NEGATIVE SPONDYLARTHROPATHY: - active and apparently painful fixation of the vertebral column - abdominal breathing - restricted flexion of the lumbar spine with a Schober test of <5 cm. - thoracic excursions of <5 cm (measured in the 3rd intercostal space). - pain on pressure in the sacroiliac joint(s) - additional systemic features such as skin, nail, or eye abnormalities
ANKYLOSING SPONDYLITIS – CLINICAL SYMPTOMS Joints Affected Most commonly the joints in the buttocks, called the sacroiliac joints, are affected. The low back is commonly involved, as is the mid-back (the thoracic spine) and the neck (the cervical spine). Of the non-spinal joints, the hips are the most commonly involved and to a lesser extent the knees and shoulders. Involvement of the small joints of the hands and feet, wrists and ankles is unusual. The joints between the ribs and the spine and between the ribs and the breast bone (sternum) can also become painful and stiff. Stiffness of these joints can result in decreased chest expansion.
ANKYLOSING SPONDYLITIS : EXTRA-ARTICULAR DISEASE Eye involvement is potentially serious with uveitis. This presents as a painful red eye. Vision may be disturbed. Up to 25% may develop uveitis. The involvement occurs especially with peripheral disease, but does not reflect severity of disease. Treatment usually requires steroid eyedrops, and is usually self limiting. Lung disease, may occur with upper lobe fibrosis, that may resemble the scarring of pulmonary Tuberculosis. Cavitation can occur of the lung parenchyma. Cardiac disease, includes, aortic valve disease, and conduction abnormalities. Cardiac enlargement may occur. Secondary amyloidosis is sometimes seen especially in the Northern hemisphere. In the Southern hemisphere for example in South Africa, it is exceedingly rare.
ANKYLOSING SPONDYLITIS - Lab Studies: • Low-grade anemia of chronic disease may be present. • Antinuclear antibody (ANA) and rheumatoid factor (RF) are within reference ranges. • Erythrocyte sedimentation rate (ESR) is normal or mildly elevated; it is more likely to be elevated with active inflammation. • C-reactive protein may be elevated with increased disease activity but is not a better indicator of inflammation than ESR. • Serum alkaline phosphatase may be elevated when active bone remodeling is occurring. • HLA-B27 antigen is positive 90-95% of the time but, notably, is not always present. Furthermore, its presence is not sufficient to make the diagnosis. Thetest is most helpful when diagnosis is not clear.
ANKYLOSING SPONDYLITIS RADIOGRAPHY - evidence of bilateral sacroiliitis with cloudy sclerosis and irregular joint delineation - evidence of anterior spondylitis, squaring of the vertebral bodies, and syndesmophyte formation - spondylodiscitis may occur in the course of the disease. BONE SCINTIGRAPHY COMPUTED TOMOGRAPHY
NEW YORK CRITERIA FOR ANKYLOSING SPONDYLITIS • Limitation of motion of the lumbar spine in all three planes (anterior flexion, lateral flexion, and extension • History or the presence of pain at the dorsolumbar junction or in the lumbar spine • Limitation of chest expansion to 2,5 cm or less, measured at the level of the fourth intercostal space THE DIAGNOSIS OF AS REQUIRES • the presence of grade 3-4 bilateral sacroilitis with at least one clinical criterion OR • the presence of grade 3-4 unilateral or grade 2 bilateral sacroilitis with criterion 1 or both clinical criteria 2 and 3
ANKYLOSING SPONDYLITIS - TREATMENT NSAID DMARD Bisphosphonates are given in short bursts over a period of a few weeks – an example is the drug called pamidronate. You may feel pain relief in the spine soon after receiving this drug. Biological therapies are drugs which are also given by injection – either as an infusion over a few hours or as a twice-weekly injection. You may also feel early benefit with these drugs. Examples are infliximab and etanercept. Exercise is one of the cornerstones to the successful long-term management of AS. It is done for three major reasons: • to maintain or restore spinal mobility, • to maintain or improve posture, • to maintain chest expansion.
PSORIATIC ARTHRITIS • Psoriasis is a relatively common skin condition characterized by increased turnover of the skin to produce plaques of keratinized scales and nail changes. • Common skin sites include the flexures of the knees, elbows, umbilicus, behind the ears and at the scalp lining - anteriorly and posteriorly. • Nail changes include pitting and ridging of the nails and are common. • In fact 5-8% of psoriasis patients develop an arthritis. • The arthritis is preceded by the rash in 70-80% of cases, but in 10 % precedes the rash. • The delay in developing the rash may be months to years. In approximately 15% the rash and arthritis appear at the same time. • The severity of the rash does not mirror the severity of the arthritis and a flare of the rash does not necessarily coincide with an arthritis flare. • Family history is common
PSORIATIC ARTHRITIS Nail changes are present in 80% of patients with the arthritis versus 20% without arthritis. The arthritis is usually seen at age 30-40’s, but it can rarely be seen in juvenile patients.There is no sex difference in incidence. There are five varieties of the arthritis but overlaps can occur: 1. Peripheral type - involves the distal interphalyngeal joints. 10% 2. Peripheral symmetrical polyarthritis - Similar to Rheumatoid arthritis. 25% 3. Asymmetrical joint type - asymmetrical, pauciarticular and affecting the hands, and feet, with sausage like digits. Large joints can be involved as an oligo/pauciarticular arthritis 80% 4. A spondyloarthropathy - affecting spine and sacroiliac joints. 5%-20% 5. A severe mutilating peripheral type – Arthritis mutilans.
PSORIATIC ARTHRITIS- CLINICAL MANIFESTATIONS Enthesitis - inflammation of tendon insertions occurs commonly, i.e. Achilles’ tendonitis. The sausage like swelling of the digits is a common characteristic manifestation of the arthritis and is caused by dactylitis. These can be seen also in reactive arthritis. Systemic involvement can occur with: Ocular changes in 30%: Conjunctivitis, Episcleritis, Keratoconjunctivitis sicca Aortic Valve disease has been described. Because of the high skin turnover - hyperuricaemia and gout can occur coinciding with the psoriasis. Association with HIV infection is also more recently identified with a tendency to severe rash and arthritis.
PSORIATIC ARTHRITIS - INVESTIGATIONS Hematology: • Elevation of the ESR and CRP are proportional to the inflammatory process of the disease. • Rheumatoid factor is negative, but in a small percentage of the population may be elevated in an unrelated and coincidental manner. Radiology : asymmetrical joint involvement, marginal erosions, widening of the joint margin, erosion of the distal tufts of the digits, subchondral erosions and development of the pencil in cup deformity, periostitis, osteolysis of the bone with telescoping of the digits, calcification of the enthesitis points with spur formation, sacroiliitis in some patients often asymmetrical, syndesmophytes as in spondyloarthropathies
ŁUSZCZYCOWE ZAPALENIE STAWÓWKRYTERIA DIAGNOSTYCZNE WG. MOLLA I WRIGHTA (1973) • obecność łuszczycy • wywiad w kierunku łuszczycy skóry i paznokci w rodzinie • zapalenie stawów • kliniczne objawy zapalenia stawów krzyżowo-biodrowych lub kręgosłupa • nieobecność czynnika reumatoidalnego
PSORIATIC ARTHRITIS - THERAPY 1. Methotrexate. 7.5 - 15 mg./ week. Dose can be increased if required up to 20-30mg / week. 2. Gold Salts - either oral or injectable. This generally helps 50-75 % of patients 3. Salazopyrine (Sulphasalazine) This I find extremely useful for the spondyloarthropathy in particular, but benefit to the arthritis as well as to the skin manifestations are reported in several studies. The dose is 1g twice daily. 4. Antimalarials are generally not used as they are frequently identified with a deterioration in the psoriasis. 5. Retinoids i.e. etretinate are used mainly for the psoriasis rash, but have been shown to also relieve the arthritis. However they have a high side effect profile and can cause teratogenicity, making their use difficult in a population of patients in childbearing years. They also interestingly have been associated with development of a hypertrophic skeletal spinal disorder - DISH - diffuse idiopathic skeletal hyperostosis.
PSORIATIC ARTHRITIS - THERAPY 6. Cyclosporine A has also been used in resistant cases at 5mg / kg / day , with improvement at 2-4 weeks. The arthritis and skin, benefit but the disease activity returns within 6 weeks of drug withdrawal. The drug needs monitoring because of hypertension and renal problems. 7. PUVA - Photo chemotherapy (Psoralen Ultraviolet A, PUVA) PUVA treatment is of benefit for some Psoriatic arthritis patients. This benefits skin and was shown to help the peripheral ( but not the axial ) arthritis as well. Surgery The use of surgery may be required in the event of joint mechanical changes and the principals of surgery are the same as for Rheumatoid arthritis The use of these drugs and therapeutic options requires experience and understanding of the potential complications. Therefore Rheumatologist involvement should be strongly considered
ENTEROPATHIC ARTHROPATHIES • Enteropathic arthropathies comprise a collection of rheumatologic conditions that share a link to GI pathology. • These conditions include reactive (ie, infection-related) arthritis caused by bacteria and spondyloarthropathies associated with inflammatory bowel disease (IBD). • Other conditions and disorders include intestinal bypass (jejunoileal), arthritis, celiac disease, Whipple disease, and collagenous colitis.
ENTEROPATHIC ARTHROPATHIES - Pathophysiology The precise causes of enteropathic arthropathies are unknown. Inflammation of the GI tract may increase permeability, resulting in absorption of antigenic material, including bacterial antigens. These arthrogenic antigens may then localize in musculoskeletal tissues (including entheses and synovium), thus eliciting an inflammatory response. Alternatively, an autoimmune response may be induced through molecular mimicry, in which the host's immune response to these antigens cross-reacts with self-antigens in synovium.
ENTEROPATHIC ARTHROPATHIES • Frequency: In the US: In patients with IBD, the prevalence of peripheral arthritis and/or sacroiliitis/spondylitis is 10-20%. • Race: Incidence of IBD is higher in whites, especially those of Jewish descent, than in other racial groups. • Sex: No sexual predilection exists in peripheral arthritis of UC or CD. The incidence of spondylitis is higher in males than females. • Age: Peak incidence of IBD occurs in persons aged 15-35 years.
