Israel Medical Association Journal

Inflammatory myopathies include polymyositis, necrotizing autoimmune myositis, dermatomyositis, juvenile inflammatory myopathy, and inclusion body myositis. These diseases are classified based on the different clinical and pathological characteristics unique to each of them [1]. Dermatomyositis is a rare disease with an incidence of 6–10 cases/1,000,000 a year with the highest incidence in the 7th decade of life as reported by a Norwegian cohort in a Caucasian population [2].

Diagnosis of dermatomyositis is based on typical signs and symptoms combined with laboratory results, imaging, and electromyography findings and muscle biopsy. Historically, the diagnosis of dermatomyositis was based on the classification criteria named after Bohan and Peter published in 1975. Many other classification criteria were proposed subsequently, the latter by the European League Against Rheumatism/American College of Rheumatology (EULAR/ACR), which were published in 2020 [3].

The clinical features of dermatomyositis are diverse. Skin manifestations can accompany or precede muscle weakness. Classical skin findings include periorbital heliotrope rash and a rash of the upper chest, back, and shoulders, known as the V sign and shawl sign respectively, as well as the Gottron's papules on the knuckles. Another skin appearance is subcutaneous calcifications that break periodically through the skin causing ulcerations. Dermatomyositis usually manifests as a symmetrical proximal muscle weakness but can present with preserved strength called amyopathic dermatomyositis [1].

Paraneoplastic syndromes are reported in 8–15% of patients diagnosed with cancer [1]. They are defined as syndromes that occur due to an underlying malignancy, which has yet to be diagnosed, or at the time of the diagnosis and less frequently following the diagnosis of a malignancy. Several mechanisms are involved including autocrine and paracrine mediators, hormones, peptides, cytotoxic lymphocytes, and cytokines [1,2].

A 42-year-old healthy man collapsed suddenly in the street while walking. The patient received 2 minutes of basic life support until an automatic external defibrillator was brought and detected ventricular fibrillation (VF), which was successfully terminated by a single shock. The patient regained consciousness and was transferred to the hospital.

The patient’s physical examination was normal with no neurologic deficit. Blood pressure was 147/102 mmHg. Brain computed tomography showed normal findings. The first troponin I measurement within 1 hour of the event was in the normal range (19.6 ng/L, normal < 20 ng/L) and rose to 99.9 ng/L after 3 hours.

Background: Statin-induced myalgia is defined as muscle pain without elevation of serum creatine phosphokinase levels and is a well-known complaint among statin users. Chronic pain syndromes affect a high percentage of the population. These pain syndromes may confound the reports of statin-induced myalgia.

Objectives: To compare the occurrence of chronic pain among patients on statin therapy who developed myalgia with those who did not.

Methods: This study included 112 statin-treated patients, who were followed at the lipid center at Sheba Medical Center. Fifty-six patients had a diagnosis of statin-associated muscle symptoms (SAMS) and 56 did not. Verified questionnaires were used to assess the diagnoses of fibromyalgia, pain intensity, functional impairment, anxiety, and depression in the study population.

Results: Patients with statin myalgia were more likely to fulfil the diagnostic criteria for fibromyalgia than patients without statin myalgia (11 [19.6%] vs. 0, respectively). Patients in the SAMS group exhibited higher levels of anxiety and depression compared with the control group. Female sex, higher scores on the Brief Pain Inventory pain intensity scale, and a Hamilton rating scale level indicative of an anxiety disorder were found to be significant predictors for fibromyalgia in patients presenting with statin myalgia.

Conclusions: A significant percentage of patients diagnosed with statin myalgia fulfilled the diagnostic criteria for fibromyalgia depression or anxiety disorder. Detection of these patients and treatment of their primary pain disorders or psychiatric illnesses has the potential to prevent unnecessary cessation of effective statin therapy.

Background: With advances in myelodysplastic syndromes (MDS), patient cohorts from different time periods might be different.

Objectives: To compare presentation and outcomes between two cohorts.

Methods: Data were collected from George Washington University Medical Center, Washington, DC, USA 1986–1987 (DC), and Tel Aviv Medical Center, Israel 1999–2009 (TA).

Results: The study comprised 227 patients (139 TA, 88 DC). TA patients were older (75.4 ± 9.8 vs. 63.8 ± 14.3 years, P < 0.001) and had more cardiovascular diseases (56.8% vs. 14.8%, P < 0.001), fewer cytopenias (1.67 ± 0.82 vs. 2.0 ± 0.93, P = 0.003), and lower mean corpuscular volume (94.3 ± 9.9 fl vs. 100.5 ± 15.3 fl, P < 0.001). Hemoglobin, leukocyte, neutrophil, and platelet counts were similar. More TA patients had dysplasias. Bone marrow cellularity and cytogenetics were similar, but more TA patients had blasts < 5% (73.4% vs. 50.6%, P = 0.003). More TA patients had early French-American-British (FAB) disease (66.9% vs. 40.9%, P < 0.001) and lower risk disease per International Prognostic Scoring System (81% vs. 50%, P < 0.001). The 5 year survival (5YS) of TA patients was not significantly greater (62% vs. 55%). 5YS by FAB was also slightly greater for TA patients (77% vs. 65% for early FAB; 43% vs. 37% for advanced FAB, P > 0.05).

Conclusions: Although patients diagnosed with MDS at a later period were older and had more cardiovascular co-morbidities, they had fewer cytopenias, tended to have earlier disease, and had minimally greater, but not significant, 5YS.

Background: Echocardiographic ventricular function predicts prognosis and guides management in patients with acute coronary syndromes. In elderly patients, interpretation of echocardiographic measurements may be difficult, especially regarding assessment of diastolic left ventricular function.

Objectives: To examine the usefulness of echocardiographic systolic and echocardiographic diastolic LV[1] function measurements as predictors of long-term outcome in elderly patients with ACS[2].

Methods: We studied 142 consecutive elderly patients (≥ 70 years old, mean age 80 ± 6 years) with ACS who had an echocardiogram at index hospitalization and were in sinus rhythm. LV ejection fraction and diastolic mitral inflow pattern were examined as predictors of survival and repeat hospitalization over a period of 18–24 months.

Results: During the 2 year mean follow-up period 35/142 patients died (25%). Survival was lower in patients with EF[3] < 40% (n=42) as compared to EF ≥ 40% (n=100) (2 year survival rate 61% vs. 81%, P = 0.038). Patients with severe diastolic dysfunction (a restrictive LV filling pattern, n=7) had a lower survival rate than those without (43 vs. 76%, P = 0.009). The most powerful independent predictor of mortality was a restrictive filling pattern (hazard ratio 4.6, 95% confidence interval 1.6–13.5), followed by a clinical diagnosis of heart failure on admission and older age. Rate of survival free of repeat hospitalization was low (33% at 18 months) but repeat hospitalization was not predicted either by EF or by a restrictive filling pattern.

Conclusions: As in the young, echocardiographic measurements of systolic and diastolic LV function predicted long-term survival in elderly patients with ACS. A restrictive filling pattern was the strongest independent predictor of mortality.

Objectives: To determine the prevalence of abnormal coagulation studies in ED[1] patients evaluated for suspected ACS[2], and to investigate whether abnormal international normalized ratio/partial thromboplastin time testing resulted in changes in patient management and whether abnormal results could be predicted by history and physical examination.

Methods: In this retrospective observational study, hospital and ED records were obtained for all patients with a diagnosis of ACS seen in the ED during a 3 month period. ED records were reviewed to identify all patients in whom the cardiac laboratory panel was performed. Other data included demographics, diagnosis and disposition, historical risk factors for abnormalities of coagulation, ED and inpatient management, INR[3]/PTT[4], platelet count and cardiac enzymes. Descriptive statistical analyses were performed.

Results: Complete data were available for 223 of the 227 patients (98.7%). Of these, 175 (78.5%) patients were admitted. The mean age was 64.2 years. Thirteen patients (5.8%) were diagnosed with acute myocardial infarction. Of the 223 patients, 29 (13%) and 23 (10%) had INR and PTT results respectively beyond the reference range. Seventy percent of patients with abnormal coagulation test results had risk factors for coagulation disorders. The abnormal results of the remaining patients included only a mild elevation and therefore no change in management was initiated.

Conclusions: Abnormal coagulation test results in patients presenting with suspected ACS are rare, they can usually be predicted by history, and they rarely affect management. Routine coagulation studies are not indicated in these patients.