What is A 65 yr old man with H/O back pain since 3 months. ESR is raised. On examination marked stiffness and mild restriction of chest movements is found. On x-ray, syndesmophytes are present in vertebrae. Diagnosis is and explain in detail?
Ans. is 'a' i.e. Ankylosing spondylitis The clinical profile of the given case fits into Ankylosing spondylitis (A.S.) except for the age.A.S. is usually seen in 2nd or 3rd decades of age with a median age of 23. However "in 5% of patients, symptoms begin after 40 " - HarrisonAll other features i.e. raised ESR, marked stiffness, restriction of chest movements & syndesmophytes are seen in A.S.SyndesmophytesSyndesmophytes are thin, slender vertical radiodense spicules bridging the vertebral bodies, giving the spine 'bamboo9 appearance. They are actually ossification of outer fibers of annulus fibrosus.Syndesmophytes are seen in:Ankylosing spondylitisPsoriatic arthritisReiter's diseaseArthritis Assoc. with Inflammatory bowel disease Before discussing A.S. lets see other options:Diffuse idiopathic skeletal hyperostosis (DISH):Also k/a Forestier's disease, ankylosing hyperostosisDISH is a syndrome of excessive calcification and ossification of soft tissues, mainly ligaments and entheses, that develops with aging. There is a marked predilection to the axial skeleton, particularly the thoracic spine. It may also involve the peripheral joints.The most characteristic radiographic abnormality of the DISH is calcification and ossification of the anterior longitudinal ligament of the spine.The diagnosis is made by spinal radiographs that show "flowing" calcifications along the anterior and lateral portion of at least four contiguous vertebral bodies without loss of disc height and without typical radiographic findings of ankylosing spondylitis, (no syndesmophytes).A DISH commonly occurs in men in their fifth to sixth decade of life.Patients are often asymptomatic but may have spinal pain or limited spinal motion.The DISH can be ruled out as in DISH there is no raised ESR and syndesmophytes. (ESR is raised in inflammatory spondyloarthropathies like Ankylosing spondylitis. But DISH and degenerative osteoarthritis are non-inflammatory conditions, so ESR would be normal.)Apley's writes- "Although DISH bears a superficial resemblance to AS, it is not an inflammatory disease; spinal pain and stiffness are seldom severe, the sacroiliitis joints are not eroded and the ESR is normal."Harrison writes- "Diffuse idiopathic skeletal hyperostosis which occurs in middle-aged and elderly is usually not symptomatic."Degenerative osteoarthritis of the spine:It is a disease of elderly characterized by the formation of large osteophytes along the anterior and lateral aspects of the spine with decreased intervertebral disc spaces.Lumbar canal stenosis:It is ruled out as - Neurogenic claudication or pseudoclaudication is the usual symptom, consisting of back and buttock or leg pain induced by walking or standing and relieved by sitting.It can be congenital (achondroplasia) or acquired in degenerative diseases, trauma, spine surgery, metabolic or endocrine disorders and Paget's disease.Let's compare the options in relation to signs and symptoms of the pt-S/s of the caseAnkylosing spondylitis(AS)Ankylosing hyper osteosis/Diffuse idiopathic skeletal hyper osteosis (DISH)Degenerative osteoarthritis of spine/spondylosis deformation65 yrs agoin 5%commoncommon3 months history+insidious long durationinsidious long durationBack pain+++/- (may be asymptomatic)+/- (may be asymptomatic)Marked stiffness++-(Stiffness is mild)|ESR+ inflammatorynon-inflammatorynon-inflammatoryRestriction of chest movements+--Syndesmophytes+"flowing wax" like calcification or hyperostoses, no | of intervertebral disc spaceOsteophytes | of intervertebral disc space! No raised ESR or syndesmophytes are seen.Ankylosing spondylitis IntroductionA prototype of seronegative (absence of rheumatoid factor) spondyloarthropathies.Inflammatory disorder of unknown cause.Usually begins in the second or third decade with a median age of 23, in 5% symptoms begin after 40.Male to female ratio is 2-3: 1Strong correlation with HLA-B27 PathologyEnthesitis i.e. inflammation of the insertion points of tendons, ligaments or joint capsule on the bone is one of the hallmarks of this entity of disease.Primarily affects axial (spinal) skeleton and sacroiliitis is often the earliest manifestation of A.S.Involvement of costovertebral joints frequently occur, leading to diminished chest expansion (normal > 5 cm)Peripheral joints for e.g. shoulders and hips (root joints) are also involved in l/3rd patients.Extraarticular manifestations like acute anterior uveitis (in 5%); rarely aortic valve disease, carditis, and pulmonary fibrosis.Pathological changes proceed in three stages-Inflammation with granulation tissue formation and erosion of adjacent bone.Fibrosis of granulation tissueOssification of the fibrous tissue, leading to ankylosis of the joint.Clinical featuresThe low back pain of insidious onsetDuration usually less than 3 monthsSignificant morning stiffness and improvement with exerciseLimited chest expansionDiffuse tenderness over the spine and sacroiliac jointsLoss of lumbar lordosis increased thoracic kyphosisDecreased spinal movements (especially extension) in all directions.Radiological featuresCardinal and earliest sign is erosion and fuzziness of the sacroiliac jointsSquaring of vertebral bodiesSyndesmophytes (described earlier) giving the appearance of a "bamboo spine".Other investigationsESR and CRP are usually elevatedHLA-B27 is present in 95% of cases.TreatmentGeneral measures to maintain satisfactory posture and preserve movement.NSAIDsTNF inhibitors for severe diseaseOperations to correct deformities ComplicationsSpinal fracturesHyperkyphosisSpinal cord compressionLumbosacral nerve root compression