A 35-year old man comes to the physician because of dyspnea on exertion over the last 3-4 months. The dyspnea is more prominent when he plays tennis. He has no symptoms at rest. He denies chest pain, palpitations, or leg swelling. His other medical problems include chronic low back pain and gastroesophageal reflux disease. He has had local corticosteroid injections due to frequent left heel pain. His current medications include ranitidine and ibuprofen daily, and occasional acetaminophen/codeine for back pain.
His blood pressure is 120/55 mm Hg, pulse is 56/min, and respirations are 12/min. He is in no acute distress. The lungs are clear to auscultation bilaterally. His apical impulse is slightly displaced both inferiorly and laterally. There is a normal S1 and S2. An early decrescendo diastolic murmur is heard best at the left upper sternal border. A third heart sound is present. His pulses are 3+ and symmetric. There is no peripheral joint inflammation or tenderness; range of motion is normal. There are no skin rashes.
Chest x-ray shows normal lung fields, a moderately enlarged cardiac silhouette, and widening of the mediastinum. The chest CT shows a dilated aortic root (4.4 cm) without evidence of dissection.
Which of the following is the most likely diagnosis?

Question

A 35-year old man comes to the physician because of dyspnea on exertion over the last 3-4 months.  The dyspnea is more prominent when he plays tennis.  He has no symptoms at rest.  He denies chest pain, palpitations, or leg swelling.  His other medical problems include chronic low back pain and gastroesophageal reflux disease.  He has had local corticosteroid injections due to frequent left heel pain.  His current medications include ranitidine and ibuprofen daily, and occasional acetaminophen/codeine for back pain.
His blood pressure is 120/55 mm Hg, pulse is 56/min, and respirations are 12/min.  He is in no acute distress.  The lungs are clear to auscultation bilaterally.  His apical impulse is slightly displaced both inferiorly and laterally.  There is a normal S1 and S2.  An early decrescendo diastolic murmur is heard best at the left upper sternal border.  A third heart sound is present.  His pulses are 3+ and symmetric.  There is no peripheral joint inflammation or tenderness; range of motion is normal.  There are no skin rashes.
Chest x-ray shows normal lung fields, a moderately enlarged cardiac silhouette, and widening of the mediastinum.  The chest CT shows a dilated aortic root (4.4 cm) without evidence of dissection.
Which of the following is the most likely diagnosis?

Quizplus · Accepted Answer

11ec193a_991d_b02b_853d_135ed6846d34_MD0002_00 This patient has a combination of chronic lower back pain, enthesitis, and evidence of aortic regurgitation from aortic root dilatation. Given his age (< 40) and male gender, ankylosing spondylitis (AS) is the most likely diagnosis. Enthesitis refers to inflammation at the site of tendon or ligament insertion into the bone, and is often seen in spondyloarthritides. Aortic regurgitation is suggested by 3+ pulses, wide pulse pressure, and diastolic murmur. Low back pain associated with AS often improves with exercise but not with rest. Other important manifestations include anterior uveitis, decreased chest expansion, and osteopenia. (Choice B) Rheumatoid arthritis (RA) is usually symmetrical and primarily involves the MCP and PIP joints. It rarely involves the spine except the cervical part. Rheumatoid arthritis can cause myocarditis, pericarditis, and peripheral vasculitis; however, valvular disease is extremely rare. (Choice C) Late syphilis can also cause aortic root dilatation and aortic regurgitation. Spinal involvement is extremely uncommon, and enthesitis is not seen with syphilis. In addition, tertiary syphilis is uncommon and usually occurs 15-30 years after the initial presentation of primary syphilis. (Choice D) The arthritis seen in systemic lupus erythematosus is usually migratory, symmetric, and polyarticular. The knees, carpal joints, and joints of the fingers are commonly involved, while the spine is less commonly involved. Libman-Sacks endocarditis and aortic regurgitation can be seen with SLE, but aortic root dilatation is uncommon. (Choice E) Systemic sclerosis (scleroderma) is associated with pulmonary hypertension, myocarditis, pericarditis, and conduction abnormalities. However, it is not usually associated with aortic valvular disease. Educational objective: Ankylosing spondylitis (AS) usually presents with chronic low back pain in patients < age 40 (men>>women). Extraarticular manifestations of AS include anterior uveitis, aortic regurgitation (due to aortitis or valve dysfunction), decreased chest expansion, and osteopenia. < __________ References: Cardiopulmonary manifestations of ankylosing spondylitis (http://www.ncbi.nlm.nih.gov/pubmed/21547038) New criteria for inflammatory back pain in patients with chronic back pain: a real patient exercise by experts from the Assessment of SpondyloArthritis international Society (ASAS) (http://www.ncbi.nlm.nih.gov/pubmed/19147614)

A 35-Year Old Man Comes to the Physician Because of Dyspnea