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2. Cardiac Service of Ignace Deen National Hospital, Guinea, West Africa.
3. Department of Diabetes and Metabolic Diseases at Donka National Hospital, Guinea, West Africa.
4. Department of Infectious and Tropical Diseases at Donka National Hospital, Guinea, West Africa.
5. Dermatology Department of Donka National Hospital, Guinea, West Africa.
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Summary: The objective of this study is to describe the cardiovascular manifestations of dermatomyositis in a 30-year-old male and the therapeutic management.
Observation: This is Mr.T.S, 30 years old, with no particular history, admitted for chest pain, palpitations of sudden onset, dyspnea of effort, cough, physical asthenia and myalgia. Evolving for two (2) weeks without cardiovascular risk factor.
On clinical examination, reveals a tachycardia at 200 beats/minute, TA at 120/70 mmHg, rattles crackling at the two (2) pulmonary bases, there are erythematous and macular lesions on the trunk, on the elbows and on the face. The electrocardiogram shows ventricular tachycardia at 200 beats/minute.
The biological examinations show a rhapdomyolysis with CPKs elevated to 3988UI/L, ASAT=228UI, ALAT=149IU, VS at 40mm, CRP at 62.07mg/l, protein electrophoresis shows a polyclonal hyper-gamma globulinemia, protidemia to 85 g / dl., PL12-positive soluble anti-nuclear antigen antibody, anti SSA/Ro52 antibody at 0.9.
Doppler echocardiography revealed dilated hypokinetic cardiomyopathy with impaired left ventricular systolic function (FE=37%).
Conclusion: Dermatomyositis is a non-specific systemic organ disease, rare, cardiac manifestations must be systematically sought, it can be observed at any age and complications are numerous.
Keywords: Dermatomyositis, cardiac manifestations, ventricular tachycardia, CHU Conakry
Dermatomyositis is a chronic condition that develops most often slowly over several weeks or months, but sometimes more rapidly evolves by relapses. This disease exists everywhere in the world affecting twice as many women as men.
It can occur at any age, but appears especially in children between 5-14 years and in adults between 50-60 years. It is sometimes perfectly disabling and its complications are numerous [1,2,4].
This is Mr.T.S, 30 years old, with no particular history, admitted for chest pain, palpitations of sudden onset, dyspnea of effort, cough, physical asthenia and myalgia. Evolving for two (2) weeks without cardiovascular risk factor.
On clinical examination, reveals a tachycardia at 200 beats/ minute, TA at 120/70 mmHg, rattles crackling at the two (2) pulmonary bases, there are erythematous and macular lesions on the trunk, on the elbows and on the face. The electrocardiogram shows ventricular tachycardia at 200 beats/minute.
The biological examinations show a rhapdomyolysis with CPKs elevated to 3988UI/L, ASAT=228UI, ALAT=149IU, VS at 40mm, CRP at 62.07mg/l, protein electrophoresis shows a polyclonal hyper-gamma globulinemia, protidemia to 85 g/ dl., PL12-positive soluble anti-nuclear antigen antibody, anti SSA/Ro52 antibody at 0.9.
Cardiac Doppler ultrasound revealed dilated hypokinetic cardiomyopathy with impaired left ventricular systolic function (EF=37%), coronary angiography did not show any significant lesion.
The frontal chest X-ray shows an accentuation of the bronchovascular framework with non-specific hilio-basal opacities.
The lung TDM shows a bilateral lung prevailing fibrosis in both (2) lung bases.
The respiratory functional exploration concludes in a disorder ventilatoire mixed severe in restrictive ascendancy The electromyogram in favor of a sensitive axonal polyneuropathy of dependent length.
Results
Given this clinical picture, the diagnosis of a dermatomyositis with cardiac manifestation was retained according to Taminoto criteria: Association of a specific cutaneous sign (Gottron sign), spontaneous myalgia, CPK elevation and existence an inflammatory syndrome.
The evolution was favorable under general corticosteroid therapy (prednisolone 1 mg/kg/day) and immunosuppressant (cyclophosphamide) and the ventricular tachycardia yielded in a few days Figure 1.
Figure 1 : Ventricular tachycardia observed at the ECG.
Cardiac involvement during dermatomyositis can take various forms: from simple electrical abnormalities (disturbances of rhythm or conduction) to more rare complications. Because of its asymptomatic nature, it has long been underestimated, but several studies used investigative means have shown that it actually existed in 30 to 70% of cases [3].
In our observation, we noted a ventricular tachycardia-like electrical anomaly recorded on the electrocardiogram.
Myocardial involvement was also noted as dilated cardiomyopathy with left ventricular systolic dysfunction. The latter makes it possible to detect the anomalies of the three (3) tunics (pericardium, myocardium and endocardium) including the valves.
Mr.T.S was treated for the purpose of reducing ventricular tachycardia with Amiodarone 200mg because of 6cp single dose then 2cp/d successfully.
He also received treatment for cardiac failure secondary to dilated cardiomyopathy based on an ACE inhibitor (Coversyl 5mg/d), betablocker (Carvedilol 6.25mg/d), and an antialdosterone (Aldactone 25mg/d).
The evolution was favorable during his hospitalization under steroids and immunosuppressors.
Dermatomyositis is a systemic disease, cardiac manifestations are diverse and underestimated, electrocardiogram and cardiac ultrasound should be systematic during the course of this systemic autoimmune disease to highlight cardiac abnormalities that are more often subclinical.
The authors declare that they have no competing interests.
| Authors' contributions | DDS | IIS | AAK | IIC | MMD |
| Research concept and design | √ | √ | √ | √ | √ |
| Collection and/or assembly of data | √ | √ | √ | √ | √ |
| Data analysis and interpretation | √ | √ | √ | √ | √ |
| Writing the article | √ | √ | √ | √ | √ |
| Critical revision of the article | √ | √ | √ | √ | √ |
| Final approval of article | √ | √ | √ | √ | √ |
| Statistical analysis | √ | √ | √ | √ | √ |
We would like to thank all the medical and surgical emergency department staff at Donka National Hospital.
EIC: Fabio Angeli, University of Perugia, Italy.
Received: 24-July-2018 Final Revised: 05-Sep-2018
Accepted: 08-Sep-2018 Published: 20-Sep-2018
Copyright © 2015 Herbert Publications Limited. All rights reserved.
