WCIM 2014 SEOUL KOREA 243
Poster Session
The Korean Journal of Internal Medicine Vol. 29, No. 5 (Suppl. 1)
PS 0751 Rheumatology
Erythrocyte Sedimentation Rate Elevation Predicts Pul- monary Impairment and Overall Mortality in Dermato- myositis
Dong Jin GO1, Hyun Mi KWON1, Eun Ha KANG2, Maximilian KONIG3, Jin Kyun PARK1, Eun Young LEE1, Eun Bong LEE1, Yeong Wook SONG1
Seoul National University Hospital, Korea1, Seoul National University Bundang Hospital, Korea2, Johns Hopkins University School of Medicine, USA3
Background: Interstitial lung disease (ILD) is a major cause of death in patients with dermatomyositis (DM). Patients with clinically amyopathic dermatomyositis (CADM) in particular have an unfavorable outcome due to ILD, which is often refractory to corticosteroid therapy. Here, we defi ne the utility of the erythrocyte sedimentation rate (ESR) as a powerful marker of ILD and increased overall mortality in patients with classic DM and CADM.
Methods: 114 patients with DM (28/114 with CADM) receiving clinical care from Jan- uary 2004 through June 2013 were analyzed. Patients who presented with respiratory symptoms or abnormal fi ndings on chest radiography underwent high resolution com- puted tomography (HRCT) and pulmonary function testing. ILD was diagnosed based on HRCT. Baseline ESR and C-reactive protein (CRP) determined at presentation were correlated with markers of pulmonary function.
Results: The mean age of patients at DM diagnosis was 49.4 ± 12.3 years. ILD was present in 53 (46.5%) patients. Among 28 patients with CADM, 14 (50%) had ILD. The mean ESR was signifi cantly higher in patients with ILD compared to patients without ILD (49.9 ± 23.3 vs. 34.4 ± 24.1 mm/hr, p=0.001). This difference was even more pronounced in CADM patients with ILD vs. without ILD (56.3 ± 23.8 vs. 25.4 ± 22.9 mm/hr, p=0.002). ESR was inversely correlated with forced vital capacity (r=-0.303, p=0.007) and carbon monoxide diffusing capacity (r=-0.319, p=0.006). Baseline ESR
= 30mm/hr was strongly associated with mortality (p=0.002, by log rank test), and invariably observed in patients that died during follow-up.
Conclusions: Baseline ESR elevation is associated with pulmonary impairment and increased mortality in patients with DM. Evaluating ESR should be an integral part of clinical care of DM.
PS 0752 Rheumatology
Role of Bone Scan in the Assessment of Polymyositis/
Dermatomyositis
Hyoun-Ah KIM1, Young-Sil AN2, Ju-Yang JUNG1, Chang-Hee SUH1 Ajou University School of Medicine, Korea1, Ajou University School of Medicine, Korea2
Background: Previous studies have reported the results of bone scan in infl ammatory myositis patients; however, they are not consistent. Therefore, in this study, we inves- tigated the clinical signifi cance of bone scan with a quantitative method for the global assessment of uptake in the proximal muscles in Korean PM/DM patients.
Methods: The 26 PM/DM patients fulfi lled the previously proposed criteria for either defi nite or probable PM/DM. The patients had undergone bone scan. The results were retrospectively analyzed visually and quantitatively using uptake ratios. The correlation between clinical parameters (laboratory and manual muscle test [MMT]) representing disease activity and fi nding of bone scan was assessed.
Results: Visual analysis determined that 10 (71.5%) active PM/DM patients had ab- normal muscle uptake in bone scan. Visual grade of bone scan gave a sensitivity and specifi city value of 74% and 90.9%, respectively in assessing infl ammation of muscle.
Maximal proximal muscle uptake ratios of bone scan were significantly greater in active PM/DM patients than inactive patients (median 1.97 vs 1.02, p=0.046). Maximal proximal uptake ratio signifi cantly correlated with creatine kinase (r=0.394, p=0.046), lactate dehydrogenase (LDH, r=0.473, p=0.015), aldolase (r=.0428, p=0.029), erythro- cyte sedimentation rate (r=0.412, p=0.036), C-reactive protein (r=0.454, p=0.002), and corresponding MMT (r=-0.399, p=0.044). Mean proximal muscle uptake ratio signifi - cantly correlated with LDH (r=0.438, p=0.025) and aldolase (r=0.572, p=0.002).
Conclusions: Visual grade and maximal proximal uptake ratio on bone scan signifi cant correlated with known disease activity markers. The data suggest that bone scan may be a useful imaging technique for evaluation of PM/DM patients.
PS 0753 Rheumatology
Sporadic Inclusion Body Myositis - A Rare Genetic My- opathies Impersonator
Helena Lobo MARTINS1, Tomás Abrantes DA FONSECA2, Teresa SEQUEIRA2, Isabel ALMEIDA2, António MARINHO2, Carlos VASCONCELOS2
Centro Hospitalar Do Porto / Centro Hospitalar Do Baixo Vouga, Portugal1, Centro Hospitalar Do Porto, Portugal2
Sporadic inclusion body myositis (IBM) is classifi ed as one of the idiopathic infl amma- tory myopathies, as well as polymyositis and dermatomyositis. However, despite some histologic similarities, the clinicopathologic manifestations of IBM are clearly distinct from the other two disorders. The recognition of amyloid deposits in IBM was helpful in further differentiating this disorder from other idiopathic infl ammatory myopathies.
The authors present the case of a 65 year-old woman with a history of hypertension and vertiginous syndrome, with about 10 years of evolution of an important proximal muscle weakness interpreted as possible dystrophy, with inconclusive muscle biopsy.
Conducted physical and occupational therapy without any benefi t, having evolved to neuromyopathy with distal and asymmetrical involvement and persistence of elevated CPK. At physical examination, muscle strength has grade 4+ / 5 in all muscle proximal segments, absence of fl exor and extensor muscles against gravity in hands and feet.
No cervical, respiratory and swallowing muscles involvement. No weakness of the pelvic girdle. Hypothesized inclusion body myopathy and repeated muscle biopsy that confi rmed diagnosis. Proposed for treatment with intravenous immunoglobulins (IgIV) and low-dose corticosteroids. IBM is a rare disorder, whose proximal muscle weakness is present initially, but distal weakness may be an early feature in some patients. The clinical diagnosis of IBM is suspected on the basis of a combination of muscle weak- ness, elevated plasma levels of muscle enzymes, myopathic features observed on clin- ical electrophysiologic studies and a conclusive muscle biopsy. The authors emphasize that the initial biopsy may be inconclusive with respect to the diagnostic histopatho- logic features of IBM. Therefore, in these cases it is important to repeat the biopsy.
PS 0754 Rheumatology
Is This a Rare Case of Quetiapine Induced Dermatomy- ositis?
Eugene TEH1, Timothy GODFFREY2
Department of Medicine, Cabrini Health, Australia1, Department of Rheumatology, Cabrini Health, Austra- lia2
Quetiapine is commonly used for schizophrenia and bipolar affective disorder. An- ti-psychotics can cause acute rhabdomyolysis as part of a neuroleptic malignant syndrome or through a direct toxic effect on myocytes. However, there are no lit- eratures or case reports of quetiapine induced dermatomyositis. In this case report, we described a possible rare case of quetiapine induced dermatomyositis. Mr. J. is a 39 year old Caucasian male who is generally in good health, with past history of As- perger’s syndrome, Tourette’s syndrome, depression and attention defi cit hyperactive disorder. He was commenced on 25mg of quetiapine by his psychiatrist for Tourette’s syndrome. On day two, he developed non-itchy erythematous rash around his eyes, neck and shoulders. At day 9, his quetiapine dose was increased to 50 mg. A week later, he presented to us with swelling of his face, neck, shoulder and right arm with diffi culty using it. He did not report any constitutional symptoms (weight loss, sweats or fever), breathlessness or diffi culty walking. On physical examination, there were no signs of neuroleptic malignant syndrome. There was evidence of Shawl-sign and heliotrope rash. His right proximal limb strength was 3/5 and remaining examination of his lower limbs, cardio-respiratory and abdomen were unremarkable. An initial di- agnosis of rhabdomyolysis was made with an elevated creatinine kinase(CK) levels of 9321U/L. He had an MRI of his right arm which showed diffused patchy intramuscular oedema typical for myositis. His CK (12826U/L) increased further with no evidence of renal impairment. His muscle biopsy was conclusive of dermatomyositis. There was no radiological evidence for primary or secondary neoplasia. Vasulitic and infectious dis- ease screen were negative, and biopsy of a colonic polyp did not show any evidence of malignancy. Could this be a rare case of quetiapine induced dermatomyositis or a mere coincidence?
