Myositiden bei Systemischer Sklerose, Lupus und Sjögren-Syndrom

Zusammenfassung

Diese Übersichtsarbeit fokussiert auf die Overlap- und undifferenzierten Myopathien, welche bei der Systemischen Sklerose (SSc), dem systemischen Lupus erythematodes (SLE) und dem Sjögren Syndrom beobachtet werden. Am häufigsten findet sich ein Myositis-Overlap bei der SSc. Da eine einheitliche Definition nicht existiert, schwanken die Angaben über die Prävalenz stark. Grundsätzlich aber sind myopathische Symptome bei der diffusen SSc deutlich häufiger als bei der limitierten Unterform. Es werden 2 myopathische Muster bei der SSc beschrieben: die „einfache“ Myopathie (milde proximale Muskelschwäche, eine normale oder nur leicht erhöhte Creatinkinase (CK) und polyphasische Potenziale im EMG von normaler Dauer und Amplitude) sowie die „komplizierte“ Myopathie (mit mindestens 3 von 4 Befunden: symmetrische Schwäche, bioptischer Nachweis eines entzündlichen Infiltrates, EMG-Veränderungen, Erhöhung der Muskelenzyme im Serum). Neuere Befunde der sogenannten Blood Oxygenation Level Dependent (BOLD) Magnetresonanztomografie legen jedoch nahe, dass die bei SSc im Muskel beobachtete Mikroangiopathie zu einer dynamischen, d. h. sich erst bei Kraftausdauer manifestierenden Muskelschwäche führen könnte. Im Allgemeinen wird angenommen, dass Patienten mit “komplizierter” Myopathie und histologisch nachgewiesener Myositis im Vergleich zur „einfachen“ Mypoathie besser auf eine immunsuppressive Therapie ansprechen. Empfohlen wird eine Glucokortikosteroid-Therapie in möglichst geringer Dosierung, da Steroide eine lebensbedrohliche renale Krise triggern können. Beim SLE werden Myalgien oder eine Muskelschwäche von bis zu 70% der Patienten beklagt, schwere Paresen und Muskelatrophien sind jedoch selten. Eine Myositis, definiert als Muskelschwäche mit begleitender CK-Erhöhung und charakteristischen EMG oder inflammatorischen Biopsiebefunden, wurde bei 6,5% der Patienten beobachtet und war in ungefähr der Hälfte der SLE Patienten bereits Teil der Initialmanifestation. Die Lupus-Myositis wird als milde Erkrankung beschrieben, obwohl sie allerdings von Rezidiven mit abwechselnden Remissionen geprägt ist. Evidenz-basierte Therapieempfehlungen existieren nicht. In der Praxis hat sich jedoch neben Kortikosteroiden der Einsatz von Methotrexat oder Azathioprin bewährt. Beim Sjögren-Syndrom wird die Prävalenz der Myositis zwischen 3 und 7% beziffert, unspezifische Myalgien wurden bei einem Drittel der Patienten beobachtet. Die Muskelsymptome korrelieren beim Sjögren-Syndrom nur schlecht mit den Biopsie-Ergebnissen, welche eine sogar noch häufigere Prävalenz der Myositis nahe legen. Allerdings ist die Ausprägung der Muskelschwäche beim Sjögren-Syndrom meist relativ mild und nur langsam progredient. Bereits geringe Initialdosen von Prednison führen in aller Regel zu einer lang anhaltenden Remission mit nur geringer Rezidivrate. Einheitliche Kriterien zur Klassifikation und Verlaufsbeurteilung der Overlap-Myositis fehlen. Eine Standardisierung der diagnostischen Methoden aller involvierten Fachdisziplinen sowie eine länderübergreifende Harmonisierung der Myositis-Definitionen und der Erfassungsgrundlagen in Registern wären wichtig.

Abstract

This review focuses on overlap myositis and the undifferentiated myopathies that are found in association with systemic sclerosis (SSc), systemic lupus erythematosus (SLE) and Sjögren’s syndrome. The most common connective tissue disease that overlaps with myositis is SSc. As there is no uniform definition, however, the reported prevalences vary greatly across studies. In general, muscle symptoms are more frequent in diffuse than in limited SSc. In SSc, 2 patterns of myopathy have been described: “simple myopathy” (characterised by mild proximal muscle weakness, normal or only slightly elevated creatine kinase (CK) and polyphasic motor unit potentials of normal amplitude and duration) and “complicated myopathy” (fulfilling at least 3 of the following 4 criteria: symmetrical muscle weakness, evidence of inflammation in a muscle biopsy, electromyographic (EMG) abnormalities, and elevated serum muscle enzymes). More recent findings obtained from Blood Oxygenation Level Dependent (BOLD) magnetic resonance imaging suggest that the microangiopathy observed in muscles of SSc patients may lead to dynamic muscle weakness, which means muscle weakness that only manifests itself with endurance strength training. Patients with “complicated myopathy” and histologically proven myositis are thought to respond better to immunosuppressive treatment than “simple myopathy” patients. Treatment with glucocorticosteroids is recommended. Steroid doses should be kept to the minimum required as higher doses can trigger a potentially fatal renal crisis. Prevalence of myalgia or muscle weakness is reported in up to 70% of patients with SLE; however, severe paresis and muscle atrophy are rare. Myositis, defined as muscle weakness and simultaneous CK elevation plus characteristic EMG or inflammatory biopsy results, is observed in 6.5% of SLE patients. In around half of these, myositis was already part of the initial disease manifestation. Lupus myositis is regarded as a relatively mild complication, but has a tendency to relapse. There is no evidence based treatment recommendation for lupus myositis, but methotrexate and azathioprine have been successfully used in clinical practice. In Sjögren’s syndrome, the prevalence of myositis is estimated to range from 3 to 7%; unspecific myalgia is observed in up to one-third of patients. Biopsy results suggest an even higher prevalence of myositis, but biopsy findings correlate poorly with clinical symptoms. Muscle weakness in Sjögren’s syndrome is relatively mild and progresses slowly. Low initial doses of prednisone usually lead to a long-lasting remission with low recurrence rates. There are no uniform classification criteria for overlap myositis. Standardisation of the diagnostic methods of all disciplines involved as well as a cross-national harmonisation of myositis definitions and the basis of data collection in registry studies would be highly desirable.

Schlüsselwörter

Myositis - Kollagenosen - Overlap-Myositis - Sklerodermie - SLE

Key words

myositis - connective tissue disease - overlap myositis - systemic sclerosis - SLE

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