Ans. is 'd' i.e., Dilated cardiomyopathy Extra aicular manifestations of ankylosing spondylitis are acute anterior uveitis (in 5%); rarely aoic valve disease, carditis and pulmonary fibrosis also occur. Ankylosing spondylitis (marie-strumpell disease) Ankylosing spondylitis is a chronic progressive inflammatory disease of the sacroiliac joints and the axial skeleton. Prototype of seronegative (absence of rheumatoid factor) spondyloahropathies. 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 : 1 Strong correlation with HLA-B27 90-95% of cases are positive for HLA - B27. Joints involved in ankylosing spondylitis Ankylosing spondylitis primarily affects axial skeleton. The disease usually begins in the sacro-iliac joints and usually extends upwards to involve the lumbar, thoracic, and often cervical spine. In the worst cases the hips or shoulders are also affected. Hip joint is the most commonly affected peripheral joint. Rarely knee (Ebenzar 4th/e 593) and ankle (Apley's 9th/e 67) are also involved. Pathology Enthesitis i.e. inflammation of the inseion points of tendons, ligaments or joint capsule on 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 costoveebral joints frequently occur, leading to diminished chest expansion (normal 5 cm) o Peripheral joints e.g. shoulders, and hips are also involved in 1/3rd patients. Extraaicular manifestations like acute anterior uveitis (in 5%); rarely aoic valve disease, carditis and pulmonary fibrosis also occur. Pathological changes proceed in three stages? Inflammation with granulation tissue formation and erosion of adjacent bone. Fibrosis of granulation tissue Ossification of the fibrous tissue, leading to ankylosis of the joint.