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Časopis Neurology – Článok Severe acute dermatomyositis with respiratory muscle weakness, rhabdomyolysis, myoglobinuria and multiple autoimmune disorders: successful treatment with long-term combination immunotherapy

Neurology

Reviewed, postgraduate scietific medical journal.
Period 3x per year
1336-8621
The journal is indexed in the Slovak National Bibliography, Bibliographiia Medica Slovaca (BMS) and listed to citation database CiBaMed. All articles are reviewed. The publisher does not bear any responsibility for data and opinions of particular authors of the articles or advertisements. The articles on grey pages are company promotions or non reviewed information, an author is responsible for the content. Any reproduction of the content is allowed only with direct consent of the editorial office.
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Neurology
Neurology
Reviewed, postgraduate scietific medical journal.
Period 3x per year
Predplatné
Téma: ORIGINAL WORKS

Severe acute dermatomyositis with respiratory muscle weakness, rhabdomyolysis, myoglobinuria and multiple autoimmune disorders: successful treatment with long-term combination immunotherapy

Peter Špalek, Jozef Lukáč, Iveta Mečiarová

Dermatomyositis (DM) is an autoimmune disease. Complement activation occurs early in the disease, leading to
the formation and deposition of membranolytic attack complex on the endomysial capillaries, followed by capillary
destruction, perivascular inflammation, and perivascular atrophy. Onset of proximal and symmetrical muscle weakness
and myalgias is typically subacute (over weeks), although it can develop abruptly (over few days). Involvement
of oropharyngeal, laryngeal or masticatory muscles occurs in cca 20 % DM patients. Characteristic skin rash usually
accompanies or precedes the onset of muscle weakness. A heliotrope rash is the classic purplish discoloration of the
eyelids, often associated with periorbital edema. A macular erythematosus rash can affect the face, neck, anterior chest
and upper back. Subcutaneous calcinosis and skin ulcerations often occur in juvenile DM but are uncommon in adult DM.
Multisystem involvement may occur in DM – interstitial lung disease, cardiac, joint, gastrointestinal manifestations
and necrotizing vasculitis. In up to 10 % of adult DM patients, there may be an underlying malignancy, necessitating
a thorough workup and careful monitoring, especially during the first 4 years from disease onset. Bohan´s and Peter´s
clinical, biochemical (hyperCKemia), EMG and histopathological criteria are practical and sensitive for diagnosis of DM.
Corticosteroids are the first-line treatment. We start the treatment with intravenous methylprednisolon (1 g/day for
3-5 days) and then switch to the oral regimen with prednisone > 1 mg/kg, as a single daily dose, taken in the morning.
In patients with severe disease we start with a combined immunosuppressive treatment (prednisone and azathioprine).
From the onset is IVIg the best choice in very rapidly progressive cases with severe weakness, myalgias and evolving
secondary rhabdomyolysis. The standard dose is 0,4 g/kg/day given for 5 days. IVIg has beneficial responses also in cases
of DM refractory to corticosteroids and immunosuppressive agents. We report a 52-year-old male patient who developed
severe acute dermatomyositis with respiratory and bulbar weakness, secondary rhabdomyolysis, myoglobinuria, renal
failure and multiple autoimmune disorders (arthritis, skin ulcerations, severe gastrointestinal inflammation, anemia,
subcutaneous calcinosis). Combined immunotherapy with i.v. methylprednisolone, prednisone, immunosuppressive
agents (azathioprine, methotrexate), i.v. cyclophosphamide and repeated courses of IVIg have been needed to achieve
an improvement in muscle strength, in activities of daily living, as well as the improvements in systemic manifestations.
Long-lasting maintenance treatment with cyclosporine (150 mg daily), prednisone (20 mg daily) and IVIg (30 g in 1
month´s intervals) is necessary because of repeated disease exacerbations.

dermatomyositis, respiratory muscle weakness, rhabdomyolysis, myoglobinuria, gastrointestinal inflammation, anaemia, arthritis, combined immunotherapy, long-lasting maintenance immunosuppressive treatment
Neurológia 2015; 10(2): 79-85

Ročník 2015  Témy časopisu Neurology 2 / 2015

OVERVIEW WORKS

Daša Viszlayová, Miroslav Brozman
Ľubomír Lisý
Martin Samohýl, Ľubomír Lisý, Roman Rams, Katarína Hirošová, Diana Vondrová, Daniela Krajčová, Alexandra Filová, Jana Jurkovičová
Pavel Šiarnik, Zuzana Čarnická, Andrej Blaho, Stanislav Šutovský, Katarína Klobučníková, Branislav Kollár, Peter Turčáni

ORIGINAL WORKS

Peter Špalek, Jozef Lukáč, Iveta Mečiarová
Andrej Blaho, Stanislav Šutovský, Pavel Šiarnik, Petra Kemenyová, Peter Turčáni

Michal Minár, Peter Valkovič
Martin Hrubiško

CASE STUDIES

Melišek Martin, Rudinský Bruno, Kurinec František
Žingor Daniel, Rudinský Bruno
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CHAIRMAN OF THE EDITORIAL BOARD
doc. MUDr. Miroslav Brozman, CSc.

MEMBERS OF THE EDITORIAL BOARD
MUDr. František Cibulčík, CSc.
doc. MUDr. Eleonóra Klímová, CSc.
doc. MUDr. Pavol Kučera, PhD.
MUDr. Marian Kuchar, PhD.
doc. MUDr. Robert Mikulík,Ph.D., FESO
MUDr. Vladimír Nosáľ, PhD.
MUDr. Ľubica Procházková, CSc.
prof. MUDr. Bruno Rudinský, CSc.
doc. MUDr. Daniel Šaňák, Ph.D.
doc. MUDr. David Školoudík, Ph.D., FESO
prof. MUDr. Karel Šonka, DrSc.
doc. MUDr. Peter Špalek, PhD.
Dr. Milan R. Voško, PhD.

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Eva Stachová
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SECTIONS

OVERVIEW PAPERS
The latest knowledge on disease and disease groups aetiology, pathogenesis, diagnoses and therapy. Maximum size is 7 pages (font size 12, line spacing 1.5) with maximum 6 pictures (graphs). In case of more extensive theme elaboration it is possible to divide the paper to several parts after agreement with editorial office. Write the article with emphasis on its practical usage for neurologists.

CASE STUDY
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1. An accurate paper title, names and surnames of all authors including titles, authors` workplace. The first author address including the phone number, fax and e-mail address.
2. Summary - concise content summary in the extent maximum 10 lines (only at overview papers, case studies and Appendices - General Medicine). Write in 1st or 3rd person singular or plural (unify according the type of an article).
3. Key words - in the extent of 3-6 (only for overview papers and Appendices - General Medicine).
4. English translation: paper title, summary, key words (only at overview papers, and Appendices - General Medicine)
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If you insert pictures into a document, send also their original files in "jpg" format, create graphs in Excel and send also their original files. If you send photo documentation via post office, please, send just high-class originals. Mark each original by a number, under which it is mentioned in the text. Write in 1st or 3rd person singular or plural (unify according the type of an article).
6. Bibliography
Citations are numbered chronologically in bold, references in the text are stated by the number of citations in parentheses. Use maximum 20 citations.

Examples of citations:
1. Pitt B, et al. The effect of spironolactone on morbidity and mortality in patients with severe heart failure. Randomized Aldactone Evaluation Study Investigators. N Engl J Med 1999; 341: 709-717.
2. Stenestrand U, Wallentin L. Swedish Register of Cardiac Intensive Care (RIKS-HIA): Early statin treatment following acute myocardial infarction and 1-year survival. JAMA 2001; 285(4): 430-436.
3. LIPID Study Group. Prevention of cardiovascular events and death with pravastatin in patients with coronary heart disease and a broad range of initial cholesterol levels. N Engl J Med 1998; 339: 1349-1357.
4. Jurkovičová O, Spitzerová H, Cagáň S. Komorové arytmie a náhla srdcová smrť pri akútnom infarkte myokardu. Bratisl Lek Listy 1997; 98: 379-389.
5. Osborne BE. The electrocardiogram of the rat. In: Budden R, Detweiler DK, Zbinden G. The rat electrocardiogram in pharmacology and toxicology. Oxford: Pergamon Press 1981: 15-27.

Do not use dots after first names in citations. Do not use colon but dot after names of authors. Use semi-colon after the year of publishing, colon is before pages. If an author is one, two or three - it is necessary to state all. If there are more than three authors it is necessary to write first three and "et all".

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Which of following factors is not related to rosacea?
a. genetic predisposition
b. Scandinavian origin
c. propionibacterium acnes
d. endothelial growth factor

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Send contributions in the e-mail to the address: stachova@amedi.sk
Period 3x per year
1336-8621
The journal is indexed in the Slovak National Bibliography, Bibliographiia Medica Slovaca (BMS) and listed to citation database CiBaMed. All articles are reviewed. The publisher does not bear any responsibility for data and opinions of particular authors of the articles or advertisements. The articles on grey pages are company promotions or non reviewed information, an author is responsible for the content. Any reproduction of the content is allowed only with direct consent of the editorial office.
Predplatné
Neurology
Neurology
Reviewed, postgraduate scietific medical journal.
Period 3x per year
Predplatné