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.Anti-dsDNAQ' C.Anti-SSAQ' D.Anti Jo-1Q' E.Anti-SmKey Concept/Objective: To know the extramuscular manifestations of polymyositis associatedwith Jo-1 antibodiesPatients with polymyositis frequently have extramuscular manifestations.One of the mostcommon is pulmonary fibrosis.Almost 70% of patients with pulmonary fibrosis will havethe autoantibody Jo-1 in their serum.Anti Jo-1 is one of the antisynthetase antibodies cur-rently found only in patients with myositis.Besides pulmonary fibrosis, the antisynthetasesyndrome includes Raynaud phenomenon, polyarthritis, and, in some cases, so-calledmechanic s hands.The last condition causes cracked and fissured skin on the hands.Anti-dsDNA and anti-Sm are autoantibodies found only in systemic lupus erythematosus, anti-Scl-70 is seen in patients with scleroderma, and anti-SSA is found in patients with eithersystemic lupus erythematosus or Sjögren syndrome.(Answer: D Anti Jo-1)31.A 50-year-old woman with a 6-month history of polymyositis complains of increasing weakness.Afterdiagnosis, she was started on 60 mg of prednisone a day, which has been tapered to 30 mg/day.The ini-tial CK was 3,000 mg/dl, but 3 weeks ago it was only slightly elevated to 400 mg/dl (normal, greater thanor equal to 275 mg/dl).Examination demonstrates cushingoid facies, 4/5 proximal muscle strength, andno abnormal heart or lung findings.Current CK is 375 mg/dl.Of the following, which is the best step to take next in the treatment of this patient?Q' A.Increase prednisone to 60 mg/day and reevaluate in 2 weeksQ' B.Refer to surgery for biopsy of one of the quadriceps musclesQ' C.Decrease prednisone to 20 mg/day and reevaluate in 2 weeksQ' D.Add methotrexate, 7.5 mg/wkQ' E.Refer to physical therapy to initiate strengthening exercisesKey Concept/Objective: To be able to recognize steroid myopathyThis patient with polymyositis has evidence of steroid myopathy.There is an increasingsense of proximal weakness without any increase in the CK.The best way to determinewhether steroid myopathy is contributing to the weakness is to try a steroid taper and see 15 RHEUMATOLOGY 19if the weakness improves.If so, a second-line agent such as methotrexate would be useful,although even methotrexate may take several weeks to months to be effective.Biopsy ofthe muscle may show type 2 fiber atrophy typical of steroid myopathy, but in the settingof polymyositis, the diagnosis may be difficult to interpret.(Answer: C Decrease prednisoneto 20 mg/day and reevaluate in 2 weeks)32.A 34-year-old woman complains of weakness, fatigue, hair loss, and numbness of the fingers.Her symp-toms began 4 months ago, soon after the delivery of her second child.While visiting her mother, she sawher mother s physician for the above complaints and was found to have a CK of 600 mg/dl.She was toldto see her local physician on returning home for evaluation of possible polymyositis.On examination,blood pressure is 90/60 mm Hg; pulse is 60 beats/min; hair appears thin; lungs and heart are normal; mus-cle strength is 5/5 in both the proximal and distal groups; and Phalen testing is positive at both wrists.Of the following, which is the best test to perform next in the evaluation of this patient?Q' A.Electromyogram (EMG) studyQ' B.Sensitive TSHQ' C.AldolaseQ' D.ANA panelQ' E.Repeat CKKey Concept/Objective: To know that the differential diagnosis of polymyositis includes hypothy-roidismHypothyroidism can cause all of the symptoms experienced by this patient as well as anelevated CK.CK levels generally do not reach those seen in inflammatory myositis.Rheu-matologic manifestations of hypothyroidism include arthralgias and even joint swelling,myalgias and muscle cramps, carpal tunnel syndrome (which this patient has), and non-specific paresthesias.(Answer: B Sensitive TSH)33.A 54-year-old woman with a recent diagnosis of dermatomyositis is referred to you for further evalua-tion.She has read that dermatomyositis can be associated with malignancy.She has recently had a thor-ough physical examination, chest x-ray, stool screening for occult blood, mammogram, and pelvicexamination, all of which were deemed unremarkable.What would you recommend to this patient?Q' A.Reassure her with regards to malignancyQ' B.Set up a routine-visit schedule for screeningQ' C.Suggest that a CEA be checked todayQ' D.Suggest that a CA-125 and transvaginal ultrasound be performedQ' E.Suggest that a colonoscopy be done soonKey Concept/Objective: To understand the relationship between dermatomyositis and malignancyIt is generally accepted that patients with dermatomyositis are at increased risk of malig-nancy, the relative risk of which is approximately four to five times that of control groups.Cancers of the ovary, lung, lymphatic system, and hematopoietic system are overrepre-sented in patients with dermatomyositis.In women older than 40 years with dermato-myositis, the risk of ovarian cancer is 20 times that of the general population.Ovariantumors are notoriously hard to find early.The most recent recommendations for detectingthem in patients with dermatomyositis include a careful gynecologic examination, meas-urement of CA-125, and transvaginal ultrasound at 3- to 6-month intervals.(Answer: DSuggest that a CA-125 and transvaginal ultrasound be performed)34.A 45-year-old man is referred to you for a workup of muscle weakness.He presented 2 months ago withproximal weakness and an elevated CK of 4,000 mg/dl.A recent biopsy showed no abnormalities.Onexamination, the patient has 4/5 strength in the proximal muscles and 5/5 strength distally; otherwise,the examination is normal. 20 BOARD REVIEWOf the following, which is the best step to take next in the management of this patient?Q' A.Order an MRI of the quadriceps musclesQ' B.Order an EMG study of the proximal musclesQ' C.Refer the patient to a muscular dystrophy clinicQ' D.Repeat CK after patient has a period of restQ' E.Perform the ischemic forearm testKey Concept/Objective: To understand the use of MRI in improving the diagnostic accuracy ofbiopsyInvolvement of muscles in polymyositis is often patchy
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