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SERONEGATIVE SPONDYLARTHROPATHY

SERONEGATIVE SPONDYLARTHROPATHY. R heumatology R esearch C enter. CHARACTERISTICS. Peripheral Arthritis: Asymmetric, Lower Limb Tendency to Sacroiliitis (X-Ray) Absence: RF, RA Nodes, Extra-articular Features Familial Aggregation HLA-B27. CLASSIFICATION. Ankylosing Spondylitis

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SERONEGATIVE SPONDYLARTHROPATHY

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  1. SERONEGATIVE SPONDYLARTHROPATHY RheumatologyResearchCenter

  2. CHARACTERISTICS • Peripheral Arthritis: Asymmetric, Lower Limb • Tendency to Sacroiliitis (X-Ray) • Absence: RF, RA Nodes, Extra-articular Features • Familial Aggregation • HLA-B27

  3. CLASSIFICATION • Ankylosing Spondylitis • Reiter’s Syndrome • Arthropathy of Inflammatory Bowel Dis. • Psoriatic Arthritis • Undifferentiated SPA • Juvenile AS

  4. ANKYLOSING SPONDYLITIS RheumatologyResearchCenter

  5. GENERAL PATTERN • Young Male • Articular Manifestations • SI Joints • Spine • Peripheral Joints: Rhyzomelic • Extra-Articular Manifestations • Uveitis • Aortitis • HLA-B27

  6. HISTORY • Brodie 1850 • 31 year old man, Ankylosed Spine, Uveitis • Strumpell 1884 • 2 patients, Ankylosed Spine, Hip Joints • Pierre Marie 1889 • Von Bechterew 1893 • X-Ray: SI joints 1930

  7. EPIDEMIOLOGY • Prevalence • 0.5 to 2 / 1000 • 10 to 20 / 1000 of B27 • 100 to 300 / 1000 of B27 + Family Background • Incidence • 7.3 / 100,000 / Year • Racial Distribution • B27 Related • White, African American, African, Japanese

  8. ETIOLOGY • Unknown • Strong Association with B27: Hypothesis • In Susceptible Individuals Immune Response Genetically Determined To Environmental Factors

  9. HLA-B27 • B*2705, B*2704, B*2702 Association • B*2706, B*2709 Preventive • HLA-B27 in General Population 2-10% • HLA-B27 in AS 90% • Iran (RRC) 55% - 60% • AS in HLA-B27 1-2% • AS in First Degree Relatives 10-30%

  10. FAMILIAL BACKGROUND • Siblings 10% • Twins • Monozygotic 63% • Dizygotic 12.5% • Dizygotic + B27 23% • Other Genetic Factors

  11. OTHER GENETIC FACTORS • HLA-B60 3-6 fold increase • Other Genetic Factors • Other HLA • B7-Creg, B38, B39, DR1, DR8 • Non-HLA • Chromosome 16 (Crohn), 17 (Psoriasis)

  12. ENVIRONMENTAL FACTORS • Shigella Flexneri • Reactive to Anti-B27 Antibody • Yersinia Enterocolittica • Reactive to Anti-B27 Antibody • Escherishia Coli • IgA Antibody in AS Patients • Klebsiella Pneumoniae

  13. KLEBSIELLA Pneumoniae • IgA & IgG Antibodies in AS • ELISA • Antigen Resembling B27 • Nitrogenase Enzyme • Cross-Reacting Antibodies • Anti-B27 Antibody • Bind to B27 positive Cells • Disease Manifestations

  14. SCENARIO

  15. INFECTIOUS DISSEMINATION MICRO-ORGANISM (Intra Cellular) APC – B27 T-Cell (CD8+) IMMUNE RESPONSE

  16. MOLECULAR MIMICRY MICRO-ORGANISM (Peptide Mimicking B27) IMMUNE REACTION T-Cell (CD8+) APC B27 Cells T-Cell (CD4+) ANTIBODY (anti B27) B-Cell

  17. AUTO-REACTIVE T CELLS HLA-B27 (Intra Thymus) MICRO-ORGANISM (Intra Cellular) APC – B27 Periphery Autoreactive CD8+ T-Cell T-Cell (CD8+) IMMUNE RESPONSE

  18. CLINICAL MANIFESTATIONS

  19. GENERAL PATTERN • Articular Manifestations • Central • SI Joints • Lumbar Spine • Dorsal Spine • Cervical Spine • Peripheral • Extra-Articular Manifestations • Enthesitis • Eye • Aorta • Kidney

  20. PAIN & STIFFNESS • INFLAMMATORY • Morning • > 1 hour • NOCTURNAL • Second half • Awaken • Walk

  21. SACROILIITIS • Bilateral Pelvic Pain • Buttock • Referral Pain • Physical Exam • Direct Pressure • Direct Mobilization • Indirect Mobilization • Evolution • Bony Ankylosis

  22. LUMBAR SPINE • Low Back Pain • Referral Pain • Sciatica Irradiation • Physical Exam • Limitation • Shober Test • Progression • Loss of Lordosis • Ankylosis

  23. DORSAL SPINE • Back Pain • Chondro-costal Pain • Intercostal Irradiation • Physical Exam • Limitation • Chest Expansion • Progression • kyphosis • Ankylosis

  24. CERVICAL SPINE • Neck Pain • Referral Pain • Cervico-Brachial Irradiation • Physical Exam • Limitation • Progression • Loss of Lordosis, kyphosis • Ankylosis

  25. SPINE DEFORMITY

  26. PERIPHERAL JOINTS • Rhyzomelic Joints • Hip • Shoulder • Talalgia • Large and Medium Joints • Small Joints • Sterno-Clavicular • Temporo-Mendibular

  27. EXTRA-ARTICULAR • Eye Involvement • Cardiovascular Manifestations • Pulmonary Disease • Neurological Manifestations • Renal Manifestations • Bowel Disease

  28. EYE LESIONS (Ant. Uveitis) • 25%, Unilateral, Acute Onset, B27 Related • Clinical Manifestations • Pain • Increased Lacrymation • Photophobia • Blurred Vision • Exam • Discolored Iris • Small Pupil • Progression Self Subsiding

  29. CARDIAC MANIFESTATIONS • Aortic Valve Incompetence • Ascending Aortitis • Cardiac Conduction Abnormality • Cardiomegaly • Pericarditis

  30. PULMONARY • Rare, very late onset (20 y) • Fibrosis of Upper lobes • Cough • Dyspnea • Hemoptysis • X-ray Linear or Patchy Opacities

  31. NEUROLOGICAL • Quadriplegia • Atlantoaxial Subluxation • Cervical Fracture  Dislocation • Paraplegia • Cervical or Dorsal Fracture • Coda Equina • Spontaneous • Fracture

  32. RENAL INVOLVEMENY • IgA Nephropathy • Amyloidosis

  33. BOWEL DISEASE • Enteric Mucosal Inflammation • Terminal Ileum • Colon • Asymptomatic

  34. INVESTIGATIONS

  35. LAB TESTS • Inflammatory • ESR • CRP • HLA-B27 • 90% (Iran 55%, RRC Studies) • Urinalysis • Proteinuria

  36. X-RAY • SI Joints • Spine • Peripheral Joints • Hip • Others

  37. SACROILIAC JOINT • Pseudo-Widening • Blurred Borders • Irregularity (Post Stamp Serration) • Bony Sclerosis • Progression • Bony Ankylosis

  38. SACROILIITIS

  39. SACROILIITIS

  40. SACROILIITIS

  41. SACROILIITIS

  42. SACROILIITIS

  43. SACROILIITIS

  44. SACROILIITIS

  45. SACROILIITIS

  46. SPINE • Syndesmophyte • Squaring (Romanus) • Ligament Ossification • Spondylodiscitis

  47. SYNDESMOPHYTE

  48. SYNDESMOPHYTE

  49. SYNDESMOPHYTE

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