Connective tissue diseases

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Fig. 8. Mixed connective tissue disease. A prominent inflammatory response is seen (arrow), with a degenerating fiber (arrow head)

Mixed Connective Tissue Emg Testing


Time course Onset/age

Clinical syndrome

Any muscle may be affected, although proximal muscles are more likely to be involved.

Variable, although involvement of muscle is unusual and tends to be seen more in chronic connective tissue disorders.

Can affect any age depending on the specific connective tissue disorder.

The following types of connective tissue diseases are associated with myopathy: 1) Mixed connective-tissue disease (MCTD); 2) Progressive systemic sclerosis (PSS); 3) Systemic lupus erythematosus (SLE); 4) Rheumatoid arthritis (RA); 5) Sjogren's syndrome (SS); 6) Polyarteritis nodosa (PAN); and 7) Behcet's syndrome (BS).

- MCTD and PSS. Most patients develop a progressive weakness associated with fatigue. The weakness may be associated with an inflammatory myopathy that resembles polymyositis, or may be associated with poor nutrition and disuse atrophy.

- SLE. A true inflammatory myopathy is rare in this disorder. Other causes of weakness include a vasculitic neuropathy associated with mononeuritis multiplex or an axonal polyneuropathy. Myopathy in SLE may be related to inflammation, disuse atrophy secondary to painful arthritis, or following use of medications such as corticosteroids or chloroquin.

- RA. Causes of muscle weakness include disuse atrophy secondary to arthritis pain, inflammatory myopathy, and medications including penicillamine.

- SS. Myalgia is common in this disorder, but inflammatory myositis is rare. Weakness is often due to disuse atrophy following joint pain.

- PAN. Although muscle biopsy may show evidence of vasculitis, symptomatic myopathy as a presenting disorder is rare in PAN.

- BS. Most patients present with painful calf or thigh symptoms, rather than muscle weakness. True myositis is unusual.

The immunopathogenesis of myositis with connective tissue disease is poorly Pathogenesis understood. The presence of anti-RNP antibodies, circulating immune complexes, and reduced complement levels all suggest activation of the humoral immune system.

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  • sirpa
    Can genetic testing show mctd?
    11 months ago

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