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Similar to Seronegative Spondyloarthropathies
Seronegative Spondyloarthropathies
- 1.
- 2. • The SeronegativeSpondyloarthropathies are a group of overlapping disorders that share certain clinical features and genetic associations. INTRODUCTION
- 4. What are thecommon features... • Seronegative (i.e. rheumatoid factor is absent). • Rheumatoid nodules are absent. • Frequent association of HLA-B27. • A tendency to occur in same family(familial aggregation). • Inflammatory axial arthritis, generally Sacroiliitis and Spondylitis. • Oligoarthritis generally with asymmetrical presentation. • Enthesitis (inflammation of the entheses, the sites where tendons or ligaments insert into the bone) e.g. Plantar fasciitis, Achilles tendonitis, costochondritis. • Extra-articular features, such as involvement of eyes (anterior uveitis), skin, genitourinary tract.
- 5. ANKYLOSING SPONDYLITIS • AnkylosingSpondylitis (AS) is an inflammatory disorder of unknown cause that primarily affects the axial skeleton,peripheral joints and extra- articular structures. • Marie-Strumpell’s Disease/Bechterew Disease.
- 6. Epidemiology • Males areaffected more frequently than females (estimates vary from 2:1 to 10:1). • Onset is between 15 and 25 years. • There is a strong tendency to familial aggregation and association with the genetic marker HLA-B27.
- 7. Pathology • Sacroiliitis isthe earliest manifestation of AS. • Synovitis, Pannus, Myxoid marrow, subchondral granulation tissue and marrow edema are found. • In Spine, there is inflammatory granulation tissue at the junction of annulus fibrosis and vertebral bone. • The outer annular fibers are eroded and replaced by bone, forming a syndesmophyte.
- 8. Pathogenesis • The pathogenesisof AS is thought to be immune- mediated, but there is no direct evidence for autoimmunity. There is uncertainty regarding the primary site of disease initiation.
- 9. Clinical Manifestations • Thesymptoms of the disease are usually first noticed in late adolescence or early adulthood median age being 23. • The initial presentation of AS generally occurs in the SI joints; involvement of the SI joints is required to establish the diagnosis. • SI joint involvement is followed by involvement of the diskovertebral, apophyseal, costovertebral, and costotransverse joints.
- 10. • The initialsymptom is usually dull aching pain, insidious in onset, felt deep in the lower lumbar or gluteal region, accompanied by low-back morning stiffness of up to a few hours duration that improves with activity and returns following inactivity. • Arthritis in the hips and shoulders occurs in 25–35% of patients. • Arthritis of peripheral joints other than the hips and shoulders, usually asymmetric,occurs in another30% of patients.
- 11.
- 12. • Neck painand stiffness from involvement of the cervical spine are late manifestations. • Chest expansion is measured as the difference between maximal inspiration and maximal forced expiration in the fourth intercostal space. Normal chest expansion is 5 cm.
- 13.
- 15. • Peripheral musculoskeletalinvolvement occurs in 30- 50% of all patients. The following are common: • Achilles tendinitis. • Plantar fasciitis. • At the tibial tuberosity. • Superior and inferior poles of the patella. • Iliac crests.
- 16. Extra-articular manifestations • Uveitis •Cardiovascular disease • Pulmonary disease • Renal disease • Neurologic disease • GI disease
- 17. Uveitis • Uveitis (alsocalled iritis or iridocyclitis) is the most common extra-articular manifestation of AS, occurring in 20-30% of patients. • Usually acute,unilateral and non granulomatous.
- 18. Cardiovascular involvement • Aortitisof the ascending aorta resulting in aortic valve insufficiency. • Mitral valve insufficiency . • Atrioventricular block.
- 19. Pulmonary involvement • Restrictivelung disease. • Bilateral apical pulmonary fibrosis.
- 20. Renal involvement • Amyloidosisis a very rare complication of AS in patients with severe, active, and long-standing disease.
- 21. Gastrointestinal involvement • Asymptomaticinflammation of the proximal colon and terminal ileum has been observed.
- 22. Laboratory Findings • Nolaboratory test is diagnostic of AS. • HLA-B27 is present in 90% of patients. • Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) are elevated. • Mild anemia may be present. • Patients with severe disease may show an elevated alkaline phosphatase level. • Elevated serum IgA levels are common. • Rheumatoid factor ,Anti-Cyclic Citrullinated peptide (CCP), and Antinuclear Antibodies (ANAs) are absent.
- 23. Radiographic Findings • Theearliest signs can be detected by 3-6 months after the onset. • SacroIliac Joints-Early patchy osteoporosis develop around the distal third of both the bones.Joint margins become illdefined and the joint intervals become widened.Subchondral erosions start and when multiple produce a rosary effect.
- 24. Grades of Sacroiliitis-according to the New York criteria Grade 0-Normal
- 25. • Grade 1-Suspiciouschanges at the left sacroiliac joint in the form of slightly irregular joint facets.
- 26. • Grade 2-Minimalabnormalities in the form of small erosions (black arrow) and slightly condensed bone (sclerosis)(white arrow)
- 27. • Grade 3-Manifestabnormalities in the form of erosion and sclerosis in addition to widening of the middle part of both sacroiliac joints.
- 28. • Grade 4-Totalankylosis of both sacroiliac joints
- 29. Lumbar Spine- • Theearliest change is squaring of the anterior portion of the vertebral bodies.The anterior concavity of the body is lost. • This is found initially at the upper lumbar and lower thoracic regions. • There will be loss of lumbar lordosis.
- 30.
- 31. • Paravertebral ossificationdevelops beneath the anterior longitudinal ligaments within the annulus at each level.The ossification develops vertically in contrast to those developed in the OA.Finally the appearance is of Bamboo spine.
- 32. • Cervical Spine-Extremebony bridging extends along the anterior aspect of the vertebral bodies.There will be loss of lordosis and apophyseal joints become ankylosed. • Hips-Severe Osteoporosis occurs in both sides of the joints.Erosions develop and the joint space becomes reduced.
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- 36. • To qualifyas the criterion for inflammatory back pain of axial SpA the chronic ( 3 months) back pain should have four or more of these characteristic features (1) age of onset below 40 years. (2) insidious onset. (3) improvement with exercise. (4) no improvement with rest and (5) pain at night with improvement upon getting up.
- 37. Treatment General measures • Patientsare encouraged to remain active and follow their normal pursuits as far as possible. • They should be taught to maintain satisfactory posture and to perform spinal extension exercises every day. • Swimming, dancing and gymnastics are ideal forms of recreation. • Rest and immobilization are contraindicated because they tend to increase the general feeling of stiffness.
- 39. Non-steroidal anti-inflammatory drugs •NSAIDs improve spinal pain, peripheral joint pain, and function over a short period of time (6 weeks). • They dont prevent or retard the progress to ankylosis.
- 40. TNF inhibitors • Etanercept,Infliximab,Adalimumab,Golimumab,a ndCertolizumab pegolhave all been approved by the US Food and Drug Administration (FDA) as therapies for AS and are indicated after NSAID therapy has failed.
- 41. • Infliximab(Remicade) -Adultdosage-5 mg/kg IV at 0, 2, and 6 weeks, then every 6 weeks. • Etanercept (Enbrel)-Adult dosage- 50 mg SC once weekly or 25 mg SC twice weekly; if twice weekly, doses should be given on same day or 3-4 days apart. • Adalimumab(Humira)-40 mg SC q2wk. • These therapies are generally reserved for individuals who have failed to be controlled with non-steroidal anti-inflammatory drugs.
- 42. OSTEOTOMIES OF THELUMBAR SPINE • Described by Smith-Petersen, Larson, and Aufranc in 1945. • If the flexion deformity is severe, the patient’s field of vision is limited to a small area near the feet. • Respiration becomes almost completely diaphragmatic. • Gastrointestinal symptoms like dysphagia or choking may occur. • In addition to improvement in function, the improvement in appearance made by correcting the deformity is achieved.
- 43. • The safestand most efficient position for this procedure is with the patient lying on his or her side. • This lateral position has several advantages: (1) it is easier to place the grossly deformed patient on the table. (2) the danger of injuring the ankylosed spine by pressure of the forehead against the table is eliminated. (3) the anesthesia is easier to manage because maintaining a clear airway and free respiratory exchange is less difficult, and (4) the operation is easier because any blood would flow out from the depth of the wound rather than into it.
- 44. 1.SMITH-PETERSEN OSTEOTOMY • Excellentoption for correction of smaller degrees of spinal deformity. • Bone is removed through the pars and facet joints . • Symmetrical resection is necessary to prevent creating a coronal deformity. • Approximately 10 degrees of correction can be obtained with each 10 mm of resection. • The osteotomy is closed with compression or with in situ rod contouring, and bone graft is applied.
- 46. PEDICLE SUBTRACTION OSTEOTOMY •Three Column Osteotomy. • Best suited for patients who have significant sagittal imbalance of 4 cm or more and immobile or fused discs. • An inherently safer procedure than the Smith-Petersen osteotomy because it avoids multiple osteotomies. • Typically,30 degrees or more of correction can be obtained with a single posterior osteotomy,at the level of the deformity.
- 48. EGGSHELL OSTEOTOMY • Reservedfor severe sagittal or coronal imbalance of more than 10 cm from the midline. • Requires both anterior and posterior approaches .
- 49. JOINT REPLACEMENT • Patientswith significant involvement of the hips may benefit from total hip arthroplasty. • Heterotopic bone formation may occur after total joint replacement, especially around the hip. Heterotopic bone formation can be reduced by giving NSAIDs (eg, indomethacin) or employing radiation therapy postoperatively.
- 50. REACTIVE ARTHRITIS/REITERS SYNDROME • Reactivearthritis refers to acute non-purulent arthritis complicated by an infection elsewhere in the body. • Syndrome was described by Hans Reiter in 1916. • A clinical triad of urethritis, arthritis and conjunctivitis occurring some weeks after dysentery or genitourinary infection.
- 51. Etiology • Gut pathogensinclude Shigella flexneri, Salmonella, Campylobacter species and Yersinia enterocolitica. • Lymphogranuloma venereum and Chlamydia trachomatis have been implicated as sexually transmitted infections.
- 52. Pathology • The pathologicalchanges are essentially the same as those in ankylosing spondylitis, with the emphasis on large-joint synovitis and a chronic disease course tending towards Sacroiliitis and Spondylitis.
- 53. Clinical features • Themost common age group is 18–40 years. • Can occur in children over 5 years of age and in older adults. • Men are affected more often than women (the ratio is about 10:1).
- 54. • The acutephase of the disease is marked by an asymmetrical inflammatory arthritis of the lower limb joints. • Knee, Ankle, Subtalar, Metatarsophalangeal, and toe interphalangeal joints, are most commonly involved, but the wrist and fingers can be involved as well. • Dactylitis, or "sausage digit," a diffuse swelling of a solitary finger or toe, is a distinctive feature of ReA.
- 56. • Tendo Achillestendinitis and Plantar fasciitis are common. • In males, urethritis and in females, cervicitis or salpingitis are common. • Ocular disease is common, ranging from asymptomatic conjunctivitis to an aggressive anterior uveitis. • The characteristic skin lesions, are keratoderma blenorrhagica.
- 58. • The chronicphase is more characteristic of a spondyloarthropathy,with features resembling those of ankylosing spondylitis.
- 59. Laboratory Findings • Testsfor HLA-B27 are positive in 75 per cent of patients with sacroiliitis. • ESR may be high in the active phase of the disease.
- 60. Radiographic Findings • Inearly or mild disease, radiographic changes may be confined to juxtaarticular osteoporosis. • With long-standing persistent disease, marginal erosions and loss of joint space can be seen in affected joints. • Sacroiliitis and spondylitis may be seen as late sequelae. • Sacroiliitis is more commonly asymmetric than in AS, and spondylitis, can begin anywhere along the lumbar spine. • The syndesmophytes may be asymmetric, coarse and nonmarginal.
- 61. Treatment • Most patientswith ReA benefit to some degree from high-dose NSAIDs. • Indomethacin, 75–150 mg/d in divided doses, is the initial treatment of choice, but other NSAIDs may be tried. • Majority of patients with chronic ReA due to Chlamydia benefited significantly from a 6-month course of rifampicin 300 mg daily plus azithromycin 500 mg daily for 5 days then twice weekly, or 6 months of rifampicin 300 mg daily plus doxycycline 100 mg twice daily.
- 62. • Tendinitis andother enthesitic lesions may benefit from intralesional glucocorticoids. • Topical steroids may be used for uveitis. • TNF inhibitors like Infliximab may be used in refractory cases.
- 63. PSORIATIC ARTHRITIS • Psoriaticarthritis (PsA) refers to an inflammatory arthritis that characteristically occurs in individuals with Psoriasis. • 60 per cent of those with psoriatic spondylitis or sacroiliitis have HLA-B27.
- 64. Pathology • The inflamedsynovium in PsA resembles that of RA, although with somewhat less hyperplasia and cellularity than in RA, and greater vascularity. • Unlike RA, PsA shows prominent enthesitis, with histology similar to that of the other spondyloarthritides.
- 65. Clinical Features • In60–70% of cases, psoriasis precedes joint disease. • In 15–20% of cases, the two manifestations appear within 1 year of each other. • In about 15–20% of cases, the arthritis precedes the onset of psoriasis.
- 67. • The spectrumof arthropathy associated with psoriasis is broad. • Five patterns are described (1) arthritis of the DIP joints. (2) asymmetric oligoarthritis. (3) symmetric polyarthritis similar to RA. (4) axial involvement (spine and sacroiliac joints). (5) arthritis mutilans, a highly destructive form of disease.
- 68. • Nail changes-Pittingof the fingers or toes occur in 90% of patients with PsA.
- 69. • Widespread shortening ofdigits (“Telescoping"). • Eye involvement, either conjunctivitis or uveitis, is reported in 7–33% of PsA patients.
- 70. Laboratory Findings • ESRand CRP are elevated. • About 10% of patients have anti-CCP antibodies. • Uric acid may be elevated in the presence of extensive psoriasis. • HLA-B27 is found in 50–70% of patients with axial disease, but 20% in patients with only peripheral joint involvement.
- 71. Radiographic Findings • Characteristicsof peripheral PsA include DIP involvement, including the classic "pencil-in-cup" deformity.
- 72. • Marginal proliferative erosions. •Small-joint ankylosis. • Osteolysis of phalangeal and metacarpal bone, with telescoping of digits. • Periostitis and proliferative new bone at sites of enthesitis.
- 73. • Characteristics ofaxial PsA include asymmetric sacroiliitis; compared with idiopathic AS, less apophyseal joint arthritis, fewer and less symmetric and coarse syndesmophytes.
- 74.
- 75. Treatment • In milddisease no more than topical preparations to control the skin disease and NSAIDs for the arthritis are needed. • In resistant forms of arthritis, immunosuppressive agents (methotrexate) and TNF inhibitors (infliximab, etanercept and adalimumab) have are effective. • Etanercept- 50 mg SC once weekly or 25 mg SC twice weekly; if twice weekly, doses should be given on same day or 3-4 days apart. • Adalimumab- 40 mg SC q2wk. • Infliximab- 5 mg/kg IV at 0, 2, and 6 weeks, then every 8 weeks.
- 76. ENTEROPATHIC ARTHRITIS • Bothforms of IBD, Ulcerative Colitis (UC) and Crohn's disease (CD) are associated with SpA. • Two types of involvement. 1.Peripheral Arthritis. 2.Sacroiliitis and Spondylitis.
- 77. 1.Peripheral arthritis • Peripheralarthritis occurs in about 15 per cent of patients with inflammatory bowel disease. • Typically larger joints are involved in asymmetric fashion. • Synovitis and joint erosion can occur. • Men and women are affected with equal frequency and there is no particular association with HLA- B27.
- 78. 2. Sacroiliitis andSpondylitis • This pattern is seen in about 10 per cent of patients with inflammatory bowel disease,. • HLA-B27 is positive in 60 per cent and there is an increased incidence of ankylosing spondylitis in close relatives. • Unlike the peripheral arthritis, sacroiliitis shows no temporal relationship to gastrointestinal inflammation and its course is unaffected by treatment of the bowel disease.
- 79. Laboratory Findings • Ofpatients with AS and IBD, 30–70% carry the HLA-B27 gene.
- 80. Radiographic Findings • Radiographicchanges in the axial skeleton are the same as in uncomplicated AS.
- 81. Treatment • Infliximab andadalimumab are effective for induction and maintenance of clinical remission in CD and UC. • Treatment for IBD, including sulfasalazine and related drugs, systemic glucocorticoids, and immunosuppressive drugs, are also usually of benefit for associated peripheral arthritis.
- 82. UNDIFFERENTIATED AND JUVENILE- ONSETSPONDYLOARTHRITIS • Patients who do not meet the classification criteria are included in this. • Approximately one-half of the patients with undifferentiated SpA are HLA-B27-positive. • In familial cases, which are much more frequently B27-positive, there is often eventual progression to classical AS.
- 83. • Juvenile-onset SpA,begins between ages 7 and 16, most commonly in boys (60–80%). • An asymmetric, predominantly lower-extremity oligoarthritis and enthesitis occurs. • Extraarticular features are absent.
- 84. • The prevalenceof B27 in this condition,is approximately 80%. • Many of these patients go on to develop AS in late adolescence or adulthood.
- 85. • Management ofundifferentiated SpA is similar to that of the other spondyloarthritides.