This disease is one of the autoimmune connective tissue disorders and is a muscular condition with necrotizing lesions of the transverse muscles, with various forms of skin damage, and with various visceral lesions. It can be accompanied by malignant tumors.
I. Medical history.
1. age, sex, onset and duration of the disease
2. Causes: infection, exertion, mental trauma, medication
3. Main clinical manifestations.
(2) Muscle symptoms: myalgia, the first site of muscle weakness, the degree, whether symmetry. Whether it is progressive aggravation, whether there is difficulty in lifting and squatting, stiff neck, heavy pressure, diplopia and eyelid drooping, difficulty in swallowing, hoarseness, dyspnea, etc.; (3) the sequence of the rash and muscle symptoms occurring subsequent; (4) systemic symptoms: general malaise, fever, arthralgia, Raynaud’s phenomenon, weight loss.
Systemic symptoms
4. Symptoms related to identification: mucosal symptoms, hair loss, renal and neurological symptoms, dry mouth and eyes, dry skin,.
5. History of treatment and medication.
6. History of visceral tumors.
7. Past and personal history: presence of endocrine diseases, hypertension, gastric ulcer, history of drug allergy
II. Physical examination
1. Vital signs, general condition of the body, routine examination of the heart, lungs and abdomen
2. Site and characteristics of the rash: purplish-red edematous rash centered on the upper eyelid, Gottron’s sign, skin heterochromatosis-like rash, thickening of the nail cortex, stiffening of the nail folds, capillary dilation.
3. muscle strength examination
4.Mucosal lesions
5.Superficial lymph nodes
C. Auxiliary tests
1.Routine laboratory tests: blood routine, urine routine, stool routine + OB, liver and kidney lipids, hepatitis B five items, anti-HIV, anti-HCV, RPR, ECG, chest X-ray, hepatobiliary, pancreatic and spleen ultrasound, cardiac ultrasound
2.Related tests: ANA+dsDNA, ENA, Jo-1, CRP, ESR, C3, C4, CH50, IG, blood protein electrophoresis, electromyography, cardiac enzyme profile, muscle biopsy, muscle strength test
3.Tumor screening CEA, CA series, PSA, lung cancer screening, AFP, CXR, whole body enhanced CT, gastrointestinal tract imaging, gastrointestinal endoscopy, bone marrow smear, bone marrow biopsy, whole body bone scan when necessary. Consultation with related departments, ENT, male: urology; female: gynecology.
IV. Diagnostic basis
1.Characteristic skin lesions
2, muscle weakness
3.Elevated muscle enzymes
4.Electromyography abnormalities
5.Muscle biopsy
V. Differential diagnosis
1. Connective tissue disease: lupus erythematosus, systemic scleroderma, mixed connective tissue disease, dry syndrome
2. Differentiation of muscle-related diseases: myasthenia gravis, progressive myotonic dystrophy, rheumatic polymyalgia, drug myopathy, hypokalemia.
VI. Treatment
1. General treatment: Patients in the acute stage should rest in bed, and should be given a high protein and low salt diet; patients with severe swallowing difficulties who cannot eat can be fed nasally, and attention should be paid to avoiding light.
2. Moderate amount of hormone: early application of adequate amount
3. Immunosuppressants: (1) combined with hormones to enhance the efficacy and reduce side effects; (2) hormone reduction difficulties or contraindications; (3) stabilization of renal function and reduction of renal fibrosis; (4) acute or critically ill patients.
4. Prevention of adverse reactions to hormones: vitamin supplementation, attention to electrolyte balance, protection of gastric mucosa, hypotension, hypoglycemia and anti-infection when necessary.
VII. Precautions in the course of treatment
1. During the acute period, control the disease development, avoid damage to important organs or irreversible damage, and strengthen functional exercise during the remission period to prevent muscle wasting atrophy.
2. Pay attention to the vital signs, blood, urine and stool routine, heart, liver and kidney function and blood sugar monitoring.
3. For male patients over 40 years old, systemic examination should be done for short duration of disease and heavy symptoms to detect tumor, if no tumor is found, attention should be paid to follow-up prevention.
4. During the treatment period, we should pay attention to the differentiation between dermatomyositis and steroidal myopathy and myasthenia gravis caused by hypokalemia.
Pay attention to the possible side effects caused by hormones and immunosuppressants, and pay special attention to the presence of lung infection.