The disease begins in acute or subacute way with muscle syndrome , myasthenia and myalgia, arthralgia, fever, skin lesions, dense widespread edema. After while, the disease gets relapsing course. Skeletal muscle involvement is observed in all patients as myalgia when making movements and even at rest with rising muscle weakness. The muscles of the shoulder and pelvic girdle are sealed, increased in volume, active movements are significantly violated, the patient can not sit on their own, raise the limbs and head from the pillow.
At a significant distribution of the process, patients are completely immobilized, and in severe cases, they are in a state of complete prostration. The spread of the pathological process to the facial muscles leads to a masklike face, damage of pharyngeal muscles that appears as dysphagia; involvement of intercostal muscles causes the disruption of breath, reducing lung capacity, hypoventilation and frequent pneumonias.
Polymyositis can affect the oculomotor muscles with the development of diplopia, strabismus, bilateral ptosis and etc.
Skin lesions are various - erythematous, papular, bullous rash, purpura, telangiectasia, hyperkeratosis, hyper and depigmentation. In some cases, a rash is accompanied by itching. It is characteristic and pathognomonic the presence of periorbital edema heliotrope erythema.
The other symptom is polyarthralgia during movements and limited mobility of the joints until the development of ankylosis mostly due to muscle failure. There is a damage of myocardiumwith inflammatory or degenerative nature that manifests by persistent tachycardia and labile pulse, hypotension, enlargement of the heart to the left, muffled tones, systolic murmur at the apex. At diffuse myocarditis severe heart failure can be developed. In this group of patients Raynaud's phenomenon is also observed.
Pulmonary involvement is rarely associated with the underlying disease, most often it is due to a trivial infection, to which patients are predisposed due to hypoventilation, aspiration of food due to dysphagia.
The gastrointestinal tract is involved in the process in almost half of the patients. As a rule, following symptoms can be marked such as anorexia, abdominal pain, gastroenterocolitis symptoms, hypotension of upper third of esophagus. The damage of the mucous membrane of the stomach and intestines with necrosis, edema and hemorrhages, up to severe gastrointestinal bleeding, perforation of the stomach, intestines; sometimes there are symptoms that stimulate intestinal obstruction.
Diagnosis can be estblished by laboratory studies:
- Increase of CPK, MB fraction of CPK, aldolase, LDH, ALT, AST
- Increase in the concentration of creatinine and urea
- Acceleration of ESR, increased level of C-reactive protein
- Antinuclear factor
- Specific antibodies: Jo-1, PL-12, Pl-7, KJ, OJ
Instrumental methods of research:
1. Electroneuromyography (electroneuromyographic) is an important method to confirm and clarify the severity of the muscle injury, significant for monitoring the effectiveness of therapy. It is also necessary to eliminate neurological disorders.
2. MRI and spectroscopy P - sensitive methods of detection of muscle inflammation.
3. ECG - a violation of rhythm and conduction.
4. X-ray examination of the lungs, often with high resolution CT, is necessary to identify the basal pulmonary fibrosis, and interstitial pulmonary fibrosis.
5. X-ray examination of the esophagus with barium suspension is to clarify the causes of dysphagia.
6. A biopsy of muscle tissue is needed to confirm the diagnosis; the most informative muscle biopsy is one involved in the pathological process.
Before the diagnosis of this disease it is very important to exclude cancer processes by means of tumor markers, EGD, colonoscopy, pelvic ultrasound, mammography, etc.