technetium-tc-99m-pyrophosphate and Dermatomyositis

To report a case of dermatomyositis (DM) with secondary Sjögren's syndrome (SS) and propose the clinical application of technetium-99m pyrophosphate ((99m)Tc-PYP) scan.. A 50-year-old woman had progressive proximal muscle weakness of bilateral thighs, myalgia, tea-colored urine, and exercise intolerance for 6 months. Physical examination showed malar rash, V-sign, periungual erythema, and mechanic hands. Neurological assessment showed symmetric pelvic-girdle weakness, myopathic face, waddling gait, but preserved deep tendon reflex and sensory functions. DM was diagnosed on the basis of typical rashes and serum creatinine kinase elevation (7397 IU/L). Aside from myopathic symptoms, dry eye and mouth were reported. Thorough autoantibody searches showed positive anti-SSA/Ro antibody (198 U/ml). Both Schirmer's test and sialoscintigraphy were positive, leading secondary SS as diagnosis. Initial (99m)Tc-PYP scan revealed increased radiouptake in the muscles of bilateral thighs, compatible with clinical assessment. Followup scan three months later shows abnormal but attenuated radiouptake at bilateral thighs, in the presence of nearly-complete clinical recovery.. DM with secondary SS in adult is a unique disease entity, with predominantly myopathic symptoms and satisfactory therapeutic response as its characteristics. Our serial muscle imaging studies suggest that (99m)Tc-PYP scan is at once anatomically-specific and persistently-sensitive to microstructural damages within inflammatory muscles, enabling clinician to monitor disease activity and therapeutic response.

Topics: Dermatomyositis; Female; Humans; Middle Aged; Radionuclide Imaging; Radiopharmaceuticals; Sjogren's Syndrome; Technetium Tc 99m Pyrophosphate

To determine the role of scintigraphy in the detection of skeletal and cardiac involvement in dermato- and polymyositis (DM/PM), we studied 30 patients with a confirmed diagnosis of DM/PM (23 females, 7 males; mean age: 35 years). Technetium-99m pyrophosphate (99mTc-PYP) and gallium-67 scans showed similar sensitivity, specificity and accuracy in the detection of skeletal muscle involvement when compared with serum enzymes (70%, 100% and 80%, respectively). Compared with the clinical parameters, 99mTc-PYP showed 70% and 67Ga 65% accuracy. Abnormal PYP cardiac uptake was observed in 57% of patients, whereas abnormal 67Ga cardiac uptake was seen in only 15%. Electrocardiography was abnormal in 40%, rest gated blood pool study in 23%, and chest X-ray in 13%. In conclusion, both 99mTc-PYP and 67Ga can be useful in the detection of the active phase of muscle disease. However, 99mTc-PYP seems to be more effective than 67Ga in the early diagnosis of cardiac involvement.

Topics: Adolescent; Adult; Child; Clinical Enzyme Tests; Dermatomyositis; Electrocardiography; Female; Gallium Radioisotopes; Gated Blood-Pool Imaging; Heart; Humans; Male; Middle Aged; Muscle, Skeletal; Polymyositis; Predictive Value of Tests; Sensitivity and Specificity; Technetium Tc 99m Pyrophosphate

Gastrointestinal complications are more common in children than in adults and present a serious problem with dermatomyositis. We report on a 66-yr-old man with dermatomyositis who suffered from intestinal perforation. The abdominal plain radiograph revealed only dilatation of the intestinal loops; increased radioactivity, however, was clearly demonstrated in the early 5-min and delayed 3-hr 99mTc-pyrophosphate images.

Topics: Aged; Colonic Diseases; Dermatomyositis; Diverticulitis, Colonic; Humans; Intestinal Perforation; Male; Radiography; Radionuclide Imaging; Technetium Tc 99m Pyrophosphate

99mTc-pyrophosphate musculoskeletal imaging and 99mTc-red blood cell gated blood pool imaging were performed on 10 patients with documented polymyositis/dermatomyositis. Abnormal 99mTc-pyrophosphate uptake by peripheral muscles was found in 8 patients (6 mild, 2 marked). Cardiac uptake occurred in 5 patients and was 3+ in 2 with cardiovascular symptoms. These 2 patients also had abnormal EF on gated blood pool imaging. Patients without myocardial 99mTc-pyrophosphate uptake had normal EF. Patients with myocardial 99mTc-pyrophosphate uptake had abnormal wall motion, in proportion to the degree of uptake. Response to therapy and outcome were poorer in patients with marked scintigraphic changes. These findings suggest that the magnitude of 99mTc-pyrophosphate myocardial uptake may have prognostic implications in these patients.

Topics: Adolescent; Adult; Cardiomyopathies; Child; Dermatomyositis; Female; Heart; Humans; Male; Middle Aged; Musculoskeletal System; Myositis; Papillary Muscles; Radionuclide Imaging; Technetium Tc 99m Pyrophosphate

Three patients with polymyositis had elevated serum levels of creatine kinase MB isoenzyme. The presence of this isoenzyme is used extensively to diagnose myocardial infarction, but the isoenzyme is also found in sera of patients with primary muscular and neuromuscular disorders. We studied cardiac function in two of our patients with electrocardiograms, technetium stannous pyrophosphate scanning, and technetium 99m-labeled erythrocyte gated blood pool imaging and in the third patient by postmortem examination. There was no evidence of myocardial involvement to account for the high serum levels of isoenzyme. Creatine kinase MB in the sera of patients with polymyositis does not necessarily indicate myocardial necrosis.

Topics: Adult; Creatine Kinase; Dermatomyositis; Electrocardiography; Female; Humans; Isoenzymes; Middle Aged; Muscles; Myocardial Infarction; Myocardium; Radiography, Thoracic; Radionuclide Imaging; Technetium; Technetium Tc 99m Pyrophosphate; Tin Polyphosphates