Section I. Lupus erythematosus
Lupus erythematosus is one of the classic autoimmune connective tissue diseases with systemic, chronic progressive recurrent attacks and remissions. The development of immunological techniques has enabled early mild and atypical cases to be diagnosed and treated in a timely manner, so that, except for a few severe cases or those with damage to vital organs, some cases can resolve on their own, and some only present with transient episodes, with complete disappearance of symptoms after a few months or a short course of the disease.
Etiology]
The causes of lupus erythematosus are not fully understood, and the factors that have been found to be related to the development of the disease are
1. Genetic factors: It is an important factor in the development of lupus erythematosus. According to statistics, the incidence rate can be as high as 5%-12% in people with a family history of lupus erythematosus. People with lupus erythematosus genes will develop the disease once they encounter certain triggering conditions in the environment. Black and yellow people have a higher probability of developing lupus erythematosus than white people.
2. Infection factors: The onset of SLE is associated with persistent and slow infection with certain viruses.
3. Endocrine factors: Estrogen can affect the onset of lupus erythematosus. Lupus erythematosus mostly occurs in women of childbearing age, but the gender ratio is almost the same in children and elderly patients. Lupus erythematosus often occurs in men with testicular hypoplasia. In addition, all those who suffer from lupus erythematosus have an increase in estrogen.
4. Environmental factors: They include physical factors (such as ultraviolet radiation) and chemical factors (such as drugs), which can directly induce lupus erythematosus.
5. Chemical factors: Some drugs such as methyldopa, phenytoin sodium, penicillamine, quinidine, insulin, etc., can cause drug lupus or aggravate lupus erythematosus.
Clinical manifestations
Disciform lupus erythematosus
It is the lightest form of lupus erythematosus and has a good prognosis. It mainly invades the skin and mucous membranes and rarely involves the internal organs, and about 5% can evolve into systemic lupus erythematosus or subacute cutaneous lupus erythematosus.
1. Skin lesions are commonly found on the exposed part and the face with butterfly-shaped lesions. They are followed by the lower lip, scalp, outer ear, chest, dorsal part of hands and feet. The lesions are confined to the head and face, and beyond the head and face, they are called disseminated type;
2, the typical damage is a purple-red plaque, the surface with adhesive scales, scales under the visible keratin plugs and enlarged pores, gradually the central color light atrophy depression in the shape of a disk. After healing, capillary dilation, atrophic scars, pigmentation or hypopigmentation. Mucosal lesions are gray-white vesicles, head lesions can lead to permanent hair loss;
3.Specialized lesions may be frostbite like or wart-like.
4, 5-10% can evolve into systemic type, a few can cause cancer;
Laboratory tests: a few cases are positive for low-titer antinuclear antibodies, such as elevated gamma globulin, positive rheumatoid factor, reduced leukocytes, and fast blood sedimentation.
Subacute cutaneous lupus erythematosus
1. The skin lesions have characteristic features and can be divided into two types.
(1) Circumscribed erythema;
(2) Papulosquamous type. The lesions are superficial, and there is no atrophic scar after healing;
2. 90% of lesions are monotypic, 10% of lesions of both types coexist, 10% of combined discoid lupus erythematosus lesions, 20% of combined systemic lupus erythematosus lesions.
3.Easy to occur photosensitivity, can be recurrent;
4. Systemic damage is mild, there may be muscle and joint pain, 10-20% have mild nephritis, heart and central nervous system damage is rare, this type can coexist with discoid lupus erythematosus, about 1/3 of cases meet the American Rheumatism Association’s SLE diagnostic criteria;
5. Laboratory tests: most of them have hypergammaglobulinemia, positive rheumatoid factor, 80% have positive antinuclear antibodies, 70% have specific anti-Ro (SSA) and anti-LA (SSB) antibodies, and individual complement levels are low.
C. Systemic lupus erythematosus
It is the most severe type of lupus erythematosus and affects more young and middle-aged women.
Typical skin lesions are butterfly-shaped erythema on the face, perinail erythema or distal subxiphoid erythema, finger (toe) end erythema and bleeding or discoid lupus erythematosus-like skin lesions. Oral mucous membrane erosion, ulceration, other may have photosensitivity, purpura, necrotizing vasculitis, erythema multiforme, erythema nodosum, large or blood, urticaria-like vasculitis, Raynaud’s phenomenon, lupus hair, etc. ;
2. Joint and muscle pain. It is one of the common early symptoms;
3, multi-organ involvement: can involve the kidney, heart, lungs, central nervous system and other important organs, other digestive exocrine glands (lacrimal gland, pituitary gland), eye can be involved;
4. Systemic symptoms: irregular fever, chills, fatigue, poor appetite, weight loss, etc.; generalized lymph node enlargement, 1/3 with hepatomegaly, 1/5 with splenomegaly;
5. Laboratory tests.
(1) Anemia, complete blood reduction, fast blood Shen;
(2) Decreased serum albumin, increased gamma globulin, IgG, IgM and loop immune complex, decreased total complement and C3, C4, positive rheumatoid factor;
(3) 90-95% of positive anti-nuclear antibodies, 60-70% of positive anti-ds-DNA, immunoblotting technique for extractable nuclear antigen antibodies (including anti-Sm, anti-RNP, anti-Ro (SSA), anti-LA (SSB) positive, anti-ribosomal antibodies and other tests) are helpful for the diagnosis of SLE;
(4) Characteristic changes in skin histopathology;
(5) Positive lupus band test (both normal skin and lesions);
(6) 30-60% positive for anticardiolipin antibodies;
(7) 40-70% of lupus cells in active SLE are positive;
(8) 2-15% of syphilis serologic tests are false positive.
[Diagnosis
1. All types of lupus erythematosus have more typical skin lesions.
2. Except for discoid lupus erythematosus, most of them have different degrees of systemic symptoms and systemic organ damage.
3. With specific autoantibodies and LBT positivity.
4. Histopathology is specific.
【Treatment
1. Explain the condition to the patient, enhance confidence in treatment, review regularly, pay attention to rest during the active period, avoid infection, prevent vaccination, pregnancy and surgery.
2, avoid overwork, pay more attention to rest, it is appropriate to eat high-calorie easy-to-digest food. Avoid sunlight or ultraviolet light, strong photoelectricity and X-ray exposure, take anti-light measures, avoid the use of photosensitive drugs such as sulfonamides, ketorolac, phenothiazine and other drugs.
3, internal medicine general disc-shaped people use quinine chloride or a small amount of corticosteroids, vitamin C and other treatment. For systemic, corticosteroids should be preferred, and they should be applied in sufficient amount and continuously, and immunosuppressants and rehmannia are used when necessary. Plasma replacement therapy can be considered for those with multiple organ damage, severe symptoms and poor efficacy of corticosteroids.
4.Topical therapy can be applied externally with corticosteroid ointment and quinine ointment.
5.Supportive therapy and symptomatic treatment of each organ lesion. Systemic lupus erythematosus with mainly systemic symptoms should be treated by internal medicine physicians to avoid delaying the disease.
Section II Dermatomyositis
Dermatomyositis (DM), also known as cutaneous heterochromic dermatomyositis, is one of the autoimmune connective tissue diseases and is a non-purulent inflammatory lesion mainly involving the transverse muscle with lymphocytic infiltration, with or without multiple skin lesions, and also with various visceral lesions. Cases in which only myositis is present are called polymyositis. Dermatomyositis may exist alone or overlap with other autoimmune diseases such as systemic lupus erythematosus, scleroderma, and rheumatoid.
[Etiology].
The exact etiology is not clear, but may be a viral infection, abnormal recognition of self by the body’s immune abnormality and vascular lesions, all three may also be interrelated, for example, lentiviral infection of the transverse muscle fibers can lead to changes in the antigenicity of the muscle fibers, which are mistaken by the immune system as “foreign”, resulting in vasculitis and the development of the disease.
Clinical manifestations
1. Dermatitis symptoms.
(1) Periorbital edematous purplish red spots centered on the eyelids, which can involve the face, neck and upper chest in severe cases;
(2) Gottron’s sign: keratotic patches and purplish papules on the metacarpophalangeal and elbow, knee and ankle surfaces, which may be accompanied by scaling and atrophy;
2. Heterochromatic skin changes, which are more common in chronic cases;
3.Other symptoms include thickening of the nail skin, perinail erythema, urticaria, erythema multiforme, necrotizing vasculitis, chronic ulcers, subcutaneous calcium deposits, and Raynaud’s phenomenon, reticulocutaneous cyanosis, oral ulcers, and photosensitivity in 10-20% of cases;
4, 8% of cases have only skin symptoms without myositis and normal muscle plum, called dermatomyositis without myositis or skin-type dermatomyositis.
5, myositis symptoms.
(1) The scapular band and the proximal transverse muscles of the extremities are the first to be involved also with the pharynx, larynx and esophagus muscle groups are obviously involved;
(2) Progressive muscle weakness, muscle swelling, pain, mobility disorders;
(3) common swallowing difficulties, reversal, hoarseness, difficulty in lifting and squatting, and even respiratory distress, heart failure, diplopia, joint muscle curvature, skin hardening, myasthenia gravis-like syndrome, etc.
6. Systemic symptoms: 40% may have irregular low or high fever, arthralgia, joint deformity and muscle contracture. Myocardial involvement may include arrhythmia and heart enlargement. Lung involvement may include interstitial pneumonia. Gastrointestinal symptoms may be seen as esophageal dilatation, barium retention in the pyriform fossa, and poor esophageal peristalsis. Lymph nodes, liver and spleen are enlarged, and malignant tumors may be combined in patients over 40 years of age.
Diagnosis
1, characteristic skin lesions.
2, multiple transverse myelitis, especially inflammation of the proximal muscles of the extremities is predominant.
3, elevated muscle plum and parallel to the disease activity, 24-hour urinary creatine elevation.
4, The presence of specific anti-PM-1, anti-JO-1, and anti-MI-2 antibodies with diagnostic value.
5.Electromyography shows myogenic injury.
6. Myocardial biopsy shows different degrees of muscle fiber degeneration.
Treatment
I. Treatment principles
1.General treatment: pay attention to rest during the active period, high protein and vitamin, high nutrition diet, avoid sunlight.
2, no tumor cases corticosteroid treatment is effective, and can also be treated with immunomodulators, non-steroidal anti-inflammatory drugs or anti-malarial drugs, thunderbolt preparations and Chinese medicine.
3, symptomatic treatment. Enhance support therapy.
Second, the principles of drug use
1, dermatomyositis early without tumor patients can choose the amount of commonly used hormones, or tretinoin, etc. and help muscle strength recovery. In cases without tumor complications, corticosteroid therapy is effective. Immunosuppressants, especially methotrexate intravenous drip combined with corticosteroid therapy is particularly effective in improving muscle strength, cyclophosphamide and azathioprine can also be applied. Other non-steroidal anti-inflammatory drugs, anabolic hormones such as nandrolone phenylpropionate, antimalarial drugs (such as quinoline chloride) and vitamin E can also be used as an adjunctive trial.
2, hormone effect is not satisfactory can be used with immunosuppressant MTX or cyclophosphamide, pay attention to the indications, contraindications and side effects.
3. In severe cases with fever, tumor, photosensitivity and Raynaud’s phenomenon, symptomatic treatment and immunomodulatory drug therapy should be chosen according to the condition.
4, severe cases can be intravenously supplemented with compounded amino acid injection, adenosine triphosphate, coenzyme A and energy combination.
5, physical therapy, passive exercise to prevent soft contracture in the acute phase of severe inflammation, twice a day, do not encourage active exercise; in the recovery period to encourage slow speed active exercise. Other massage, tui-na hydrotherapy, and transthermal electrotherapy can be used as appropriate to prevent muscle atrophy and contracture. Rehabilitation therapy training is provided for patients with loss of function.
Section III Scleroderma
Scleroderma is a connective tissue disease characterized by limited or diffuse fibrosis, sclerosis and atrophy of the skin and connective tissue of visceral organs.
[Etiology].
1, genetic factors: according to some patients have a significant family history, the increased incidence of HLA-B8 in patients with severe disease and chromosomal abnormalities in the relatives of patients, it is believed that the characteristics of the genetic type may be on the dominant allele of the X chromosome.
2, infection factors: many patients often have acute infections before the onset of the disease, including pharyngitis, tonsillitis, pneumonia, scarlet fever, measles, sinusitis, etc. Paramyxovirus-like inclusions have been found in the transverse muscles and kidneys of patients.
3. Abnormalities in connective tissue metabolism: Patients show extensive connective tissue lesions with significantly increased collagen content in the skin, more soluble collagen and unstable intermolecular side chains within the skin damage during the viable phase of the virus, and fibroblast cultures of patients show significantly increased activity of collagen synthesis.
4, vascular anomalies: patients have Raynaud’s phenomenon, not only limited to the extremities, also occurs in the visceral vessels; histopathology shows that the skin lesions and visceral can have small blood vessels (arteries) contracture and endothelial hyperplasia, so some people believe that the disease is a primary vascular disease, but because vascular lesions are not seen in all patients, it is also believed that vascular lesions are not the only pathogenic factors of the disease.
5, epidemic abnormalities: this is the most important view in recent years, in the patient’s body can be measured a variety of autoantibodies (such as anti-nuclear antibodies, anti-DNA antibodies, anti-ssRNA antibodies, anti-scleroderma skin extract antibodies, etc.); the patient’s body B-cell count increased, humoral immunity is significantly enhanced, in systemic patients circulating immune complex determination positive rate of up to 50% or more, most patients have hypercalcemia Most patients have hypergammaglobulinemia; some cases are often complicated with lupus erythematosus, dermatomyositis, rheumatoid arthritis, dry syndrome or Hashimoto’s thyroiditis, and most believe that this disease may be an autoimmune disease caused by persistent chronic infection on the basis of a certain genetic background.
Clinical manifestations
I. Skin lesions
It can be divided into 3 stages: edema, sclerosis and atrophy.
1, edema phase: manifested as skin thickening, tautness, wrinkles disappear, non-depressed or depressed edema, pale or yellowish color, low skin temperature, reduced sweating, small chaps on the skin surface, disappearance of fingertip fat pad, in patients with limited skin lesions early edema appears in the fingers, back of the hand and face, later spread to the upper limbs, neck, shoulders, etc., diffuse type is often first by the trunk, and then to the This period can last for several months.
2, sclerosis phase: skin thickening and hardening, fibrosis, fingers, back of the hand shiny and taut, early skin can be red, the surface has a wax-like luster, no sweating, hair sparse, skin is not easy to pinch up, facial skin involvement can appear facial tautness, expression fixed, lip thinning, radioactive grooves around the mouth, difficulty in opening the mouth, the end of the nose becomes pointed, fingers gradually thinning, the end fingers become pointed and short, ulcers can appear, can produce Skin changes can be limited to the fingers, toes, hands, feet and face, and can extend to the forearms, or start from the back of the chest and extend to the periphery, involving the upper arms, shoulders, abdomen and legs, usually within 3 years of onset the extent and severity of skin lesions reaches its peak.
3.Atrophic phase: Skin atrophy and thinning like parchment, sometimes subcutaneous tissue and muscle atrophy and sclerosis can also occur, skin lines disappear, hair loss, smooth and thin skin, close to the bones, finger ends and joints prone to stubborn ulcers, capillary dilation and subcutaneous tissue calcification can also occur.
The above is the typical skin damage and process of scleroderma, except for Sine scleroderma without skin manifestation, all other types can appear, there is no obvious boundary between these three stages, but a gradual process of skin lesions.
II. Raynaud’s phenomenon
1, scleroderma vasculopathy: almost all scleroderma patients have Raynaud’s phenomenon, suggesting that vasculopathy is the basis for the development of scleroderma, when the patient is cold or emotional excitement, there will be hand and foot finger (toe) skin capillary pre-capillary artery and arteriovenous shunt closure caused by skin pallor, followed by cyanosis changes, the end of stimulation (turn warm), the vascular spasm is lifted, the skin turns flushed, with numbness, burning, and Stinging sensation, usually takes 10-15min, fingers (toes) become normal color or spotted.
2, primary Raynaud’s phenomenon: Raynaud’s phenomenon is divided into two categories: primary (idiopathic) Raynaud’s phenomenon, also called Raynaud’s disease and secondary Raynaud’s phenomenon, the former cause is unknown, the latter is secondary to certain diseases or known causes of vasospasm.
A survey of the general population shows that 4% to 15% of people have Raynaud’s phenomenon manifestations, most of them have no changes in vascular structure or tissue ischemic damage (primary Raynaud’s phenomenon), 50% of patients with Raynaud’s phenomenon are primary, typical primary Raynaud’s phenomenon begins in adolescents, 20 to 40 years old is common, more women than men, primary Raynaud’s phenomenon patients are basically normal in other aspects, symptoms Appear in the fingers (toes), symmetrical distribution.
3, Raynaud’s phenomenon and scleroderma: 90% of patients with scleroderma have obvious Raynaud’s phenomenon, the appearance of Raynaud’s phenomenon is a manifestation of vasospasm and abnormal finger artery structure, compared with normal people, patients with scleroderma have malnutrition, as well as in the low temperature environment by temperature regulation of blood flow to the skin overreaction, the same in the environment turns warm, lifting vasoconstriction, restoration of local blood flow response is also delayed.
C. Muscle, joint and bone lesions
1, muscle lesions: scleroderma patients often show obvious myalgia, muscle weakness, which can be the earliest non-specific symptoms of scleroderma, late muscle atrophy can also appear, on the one hand, due to skin thickening and hardening to limit the motor function of joints, resulting in muscle disuse atrophy, diffuse scleroderma this situation can occur in any joint, but the fingers, wrists, elbow joints are more common, on the other hand, also related to the spread of fibrosis from tendons to When scleroderma overlaps with polymyositis or dermatomyositis the patient may have significant proximal muscle weakness, persistent increase in serum creatine phosphokinase (CPK), EMG showing increased polyphasic potentials, decreased wave amplitude and time frame, no insertional stress and fibrillation, muscle tissue biopsy showing inflammatory changes, inflammatory cell infiltration, muscle fiber degeneration, atrophy or fibrosis (Figure 12, 13).
2, arthritis: arthritis mostly occurs in the fingers, wrists, knees, ankles and other extremity joints, arthralgia can also be a non-specific symptom of early scleroderma, patients often have more pronounced joint pain and morning stiffness, pain can be involved in the upper arm and calf muscles along the tendons from the joint, wrist, ankle, elbow, knee joint pain increases when moving, and can appear rougher friction sound, which is due to fibrosis and inflammation of the tendon sheath and adjacent tissues. This is due to the fibrosis and inflammation of the tendon sheath and adjacent tissues, this friction sound is mostly seen in diffuse scleroderma, suggesting a poor prognosis, about 29% of patients can develop erosive arthropathy.
3, bone lesions: X-ray skeletal examination can be found osteoporosis, osteosclerosis, bone destruction, bone atrophy, extremity, finger plexus bone resorption resulting in thinning and shortening of the finger bones, soft tissue calcification, joint space narrowing, bone erosion and joint ankylosis is less common.
Fourth, digestive system lesions
80% to 90% of scleroderma patients can have digestive system involvement, which is also the first symptom of scleroderma.
1, oropharynx: restricted opening, dry oral mucosa, sclerosis, disappearance of tongue papillae, tongue muscle atrophy, tongue can not be extended out of the mouth, periodontal disorders lead to chewing difficulties, tooth loss and malnutrition, the general function of the epiglottis is not affected, unless the striated pharyngeal muscle is involved, myositis or neuromuscular lesions, at this time, food into the esophagus through the mouth will have difficulty swallowing, patients swallowing liquid food choking, coughing. Transnasal reflux, head, shoulder forward flexion can suggest primary myopathy or neurological disorders.
2, esophagus: 80% to 90% of patients have abnormal esophageal function, common symptoms are dysphagia, food reflux and malnutrition, dysphagia is often manifested as a feeling of food sticking in a part of the esophagus after swallowing solid food, can be relieved after drinking water, the patient’s esophageal peristalsis is weakened can appear a feeling of fullness as well as acute food impaction.
Five, lung lesions
The earliest symptom is shortness of breath after activity; later manifests as a dry cough without sputum, chest pain is usually not caused by scleroderma pulmonary lesions, but is related to skeletal muscle sarcoidosis, reflux esophagitis, pleurisy or pericarditis, patients with scleroderma have both pulmonary interstitial fibrosis and pulmonary artery vasculopathy, but with one pathology Patients with scleroderma have both pulmonary interstitial fibrosis and pulmonary artery vasculopathy, but one pathology is predominant. Pulmonary interstitial fibrosis is more pronounced and severe in patients with diffuse scleroderma who are positive for anti-Sc1-70 antibodies, while pulmonary vasculopathy and pulmonary hypertension are the main pulmonary manifestations in patients with CREST syndrome. Pulmonary arterial hypertension, right heart failure due to pulmonary hypertension, etc.
Sixth, cardiac lesions
It can be manifested as myocarditis, pericarditis or endocarditis, and the obvious clinical signs of various heart diseases suggest a poor prognosis. The main symptoms of cardiac involvement in scleroderma include shortness of breath after activity, palpitations, chest discomfort and the corresponding clinical manifestations caused by pericarditis, congestive heart failure, pulmonary hypertension and arrhythmias, and the presence of cardiac manifestations is associated with the presence of anti-Scl-70 antibodies or anti-RNA polymorphase antibodies. Patients with scleroderma can have pulmonary heart disease due to lung damage, causing right heart failure. Simple pulmonary hypertension without pulmonary fibrosis manifestations is uncommon and almost exclusively seen in CREST syndrome.
VII. Renal lesions.
Renal lesions in scleroderma are most notable in interlobular arteries, arcuate arteries and small arteries, the most prominent of which are small interlobular arteries. The intima has fibroblast proliferation, mucinous changes, acidic mucopolysaccharide deposition and edema; vascular smooth muscle cells undergo hyaline degeneration; the outer membrane and surrounding interstitium are fibrotic; the glomerular basement membrane is irregularly thickened. The clinical manifestations of scleroderma nephropathy vary, with some patients having years of skin and other visceral involvement without renal damage; some develop renal crisis during the course of the disease, i.e., sudden onset of severe hypertension and acute renal failure. If left untreated, death from heart failure and uremia often occurs within a few weeks. Although the initial phase of renal crisis may be asymptomatic, most patients experience increased fatigue, shortness of breath, severe headache, blurred vision, convulsions, and confusion. Laboratory tests reveal increased creatinine, proteinuria and/or microscopic hematuria, and there may be microvascular hemolytic anemia and thrombocytopenia.
VIII: Other clinical manifestations
1, depression: 50% of scleroderma patients have depressive manifestations, the degree of which is related to the personality characteristics of the patient and the care received, but not to the disease.
2, hypogonadism: in scleroderma patients are more common, especially in male patients usually secondary to neurovascular lesions.
3, dry eyes, dry mouth: this is a common manifestation of scleroderma patients, minor salivary gland biopsy shows fibrotic changes, but not with the characteristic lymphocyte infiltration of the Schellen syndrome, in the majority of patients also did not detect anti-SSA (Ro) antibodies and anti-SSB (La) antibodies, indicating that the mucosal dryness of scleroderma patients in the mechanism of occurrence is different from the Schellen syndrome.
4, neurological lesions: Scleroderma has no central nervous system involvement, but can involve peripheral nerves, such as trigeminal neuropathy, carpal tunnel syndrome, etc. Abnormal neurological function is often secondary to microvascular lesions, such as Raynaud’s phenomenon is related to increased adrenergic neuroreactivity, gastrointestinal manifestations are also related to impaired cholinergic nerve function, indicating that patients with scleroderma have autonomic dysfunction.
5, hypothyroidism: 25% of patients have hypothyroidism, which is related to thyroid fibrosis or autoimmune thyroiditis, patients may have anti-thyroid antibodies in the serum, pathological manifestations of lymphocyte infiltration.
6, pregnancy: Scleroderma patients often have abnormal menstruation, so the conception rate is lower than normal, the rate of spontaneous abortion, preterm birth and low birth weight babies are higher than the normal population, but this does not mean that scleroderma patients in the course of the disease can not be pregnant, usually pregnancy does not aggravate the disease of systemic sclerosis, but pregnancy can aggravate reflux esophagitis and cardiac symptoms.
【Examination
1.General laboratory tests: no special abnormalities. Blood sedimentation may be normal or mildly increased. Anemia. There may be a mild decrease in serum albumin and an increase in globulin.
2.Immunological test: The positive rate of serum ANA is over 90%, and the karyotype is mainly speckled and nucleated. In patients with CREST syndrome, about 50% to 90% are positive for anti-adhesion antibodies, while only 10% of cases in diffuse scleroderma are positive. About 20% to 40% of patients with systemic sclerosis have positive serum anti-Scl-70 antibodies. About 30% of cases are positive for rheumatoid analogues. Other patients may have anti-RNA polymorphase III antibodies and anti-fibrillar protein antibodies.
3, pathology and nail fold microcirculation examination: Sclerotic skin biopsy shows increased dense collagen fibers in the reticular dermis, thinning of the epidermis, loss of epidermal protrusions, and atrophy of skin appendages; large aggregations of T lymphocytes can be seen in the dermis and subcutaneous tissue (also at the site of extensive fibrosis). Microscopic examination of the nail fold capillaries showed dilated capillary collaterals and disappearance of normal blood vessels.
4.High-resolution CT examination: In combined interstitial lung disease, exudative lesions or fibrotic changes in the lungs or distended bronchial dilatation can be found.
5, pulmonary function tests: patients with interstitial lung disease may be found to have decreased exertional lung volume, total lung volume, and decreased carbon monoxide diffusion.
6, cardiac catheterization: as a screening test for patients with pulmonary hypertension, echocardiography can detect pulmonary hypertension, but the confirmatory method is to perform cardiac catheterization, which is the only gold standard for confirming the diagnosis of pulmonary hypertension.
[Diagnosis].
According to the diagnostic criteria of the American College of Rheumatology on systemic sclerosis in 1980, systemic sclerosis can be diagnosed if one of the following major criteria or two minor criteria are present
1. Primary criteria: There is proximal scleroderma, i.e., symmetrical thickening, tautness and sclerosis of the skin of the fingers and any part of the skin above the metacarpophalangeal or metatarsophalangeal joints. Such changes can involve the entire limb, face, neck and trunk (chest and abdomen).
2. Secondary criteria: bilateral basal lung fibrosis: standard chest radiographs show bilateral reticular linear or linear nodular shadows, most marked at the base of the lungs, which may have a diffuse ground glass or “honeycomb lung” appearance. These changes cannot be attributed to a primary lung lesion. Scleroderma of the fingers: These skin changes are limited to the fingers. Depressed scarring of the fingers or loss of finger pad tissue: ischemia-induced depression of the fingertips or loss of finger pad (finger belly) tissue.
3, CREST syndrome criteria: having three of the five items of calcification, Raynaud’s phenomenon, esophageal motility disorder, scleroderma and capillary dilation and anti-adhesion antibody can confirm the diagnosis.
Treatment
I. General treatment
1. Quit smoking to avoid causing vasospasm. Avoid the use of drugs that can aggravate the disease, such as beta-blockers. Use cotton gloves and thick socks to keep hands and feet warm, and wear more clothes to prevent reflex effects caused by cold stimulation of the trunk. Rotate the upper arm to improve blood circulation.
2, glucocorticoids and immunosuppressants: In general, glucocorticoids are not effective for this disease, but they are effective for inflammatory myopathy and inflammatory phase of interstitial lung disease; they are also effective in the early edema phase, arthralgia and myalgia. The dose of prednisone is 30-40 mg/d for 3-4 weeks and then tapered to a maintenance dose of l0-15 mg/d. There is not much research literature on the treatment of dermatosclerosis with immunosuppressive agents. Commonly used cyclosporine A, cyclophosphamide, azathioprine, etc., have been reported to have certain efficacy on skin joint and kidney lesions, and combined with glucocorticoids, can often improve the efficacy and reduce the amount of glucocorticoids.
3.Anti-fibrosis treatment (connective tissue formation inhibitor): penicillamine, in the process of conversion of procollagen into collagen, monoamine oxidase is required to participate in polymerization and cross-linking. Start with 0.125g daily, taken on an empty stomach. Generally increase 0.125 g/d for 2 to 4 weeks, with discretionary use up to 0.75-1 g/d. After 6 to 12 months of dosing, the skin may soften and the frequency of renal crisis and progressive lung involvement may decrease. Adverse drug reactions occur in approximately 47% of patients taking this drug. 29% of patients discontinue the drug as a result. Common adverse reactions include fever, anorexia, nausea, vomiting, mouth ulcers, abnormal taste, rash, leukopenia and thrombocytopenia, proteinuria and hematuria.
4.Methotrexate: The only treatment recommended by the European League Against Rheumatism’s recommended treatment guidelines for skin sclerosis is methotrexate. The results of 2 high-quality randomized controlled trials have shown that methotrexate, taken orally or by intramuscular injection, can improve the progression of skin lesions associated with systemic sclerosis and is therefore recommended for skin lesions in systemic sclerosis; the recommended dose is 10 to 15 mg once a week.
5.Other: In recent years, there are reports in the literature on the use of relaxin, imatinib, CD20 monoclonal antibody, TGF-β antibody and other new therapeutic methods for the treatment of skin sclerosis have achieved good efficacy, but they have not been widely promoted for use, and can be considered for refractory patients.
Second, symptomatic treatment
1, Raynaud’s phenomenon: quit smoking, hands and feet to avoid cold to keep warm. If the symptoms are severe and there is a tendency of necrosis, endothelin receptor antagonist bosentan or sildenafil can be added. Intravenous prostaglandin analogs may also alleviate Raynaud’s phenomenon and are used to treat finger-end ulcers. Sympathetic nerve block may be considered for gangrene of the fingers. The European League Against Rheumatism evidence-based medical treatment guidelines recommend nifedipine 10-20 mg, 3/d and the intravenous dose of the prostaglandin analogue iloprost (0.5-3 ng/kg/min for 3-5 days). In the absence of intravenous preparations, oral epoprostenol 50-150 mg, 2/d is also an option, but is less effective than intravenous preparations. In the presence of ulcers, the endothelin receptor antagonist bosentan 62.5 mg, 2/d for 4 weeks is recommended, followed by 125 mg, 2/d for 12 weeks, which can prevent the occurrence of new ulcers.
2.Gastrointestinal tract involvement
(1) Proton pump blockers: Although there is a lack of evidence from randomized controlled trial studies, experts believe that proton pump blockers (PPI) can prevent gastroesophageal reflux, esophageal ulcers and their resulting esophageal strictures associated with systemic sclerosis; therefore, they are recommended for the prevention of gastroesophageal reflux, esophageal ulcers and strictures in patients with systemic sclerosis (strength of recommendation B).
(2) Gastrointestinal motility drugs: randomized controlled trial studies have shown that cisapride improves gastric emptying and lower esophageal sphincter pressure, but cisapride has been recalled in many countries because it can cause long QT syndrome; whether other gastrointestinal motility drugs can be used for systemic sclerosis-associated gastrointestinal lesions lacks the results of randomized controlled trials, but because of its role in diseases other than systemic sclerosis, the panel believes that Gastrointestinal motility drugs can be used to treat symptoms of systemic sclerosis-associated gastrointestinal hypokinesia (strength of recommendation C).
(3) Antibiotics: Despite the lack of specific randomized controlled trial studies, the panel believes that malabsorption caused by excessive intestinal bacterial growth is beneficial with rotation of quinolones or amoxicillin-clavulanic acid (recommendation strength D).
【Prevention】.
1. Primary prevention.
①Remove infected lesions, pay attention to hygiene, strengthen physical exercise, and improve their own immune function.
② regular life, work and rest, relaxed mood, avoid strong mental stimulation.
③ Strengthen nutrition, forbid eating cold, pay attention to warm tonic.
2.Secondary prevention.
①Early diagnosis: diagnosis can be made based on typical skin sclerosis and systemic damage.
Early treatment: use glucocorticoid or its suspension for intra-dermal injection, adhere to physical therapy and physiotherapy, such as audio, wax therapy, etc., to improve limb contracture and increase limb function, or long-term oral vitamin E.
3.Tertiary prevention.
①Progressive systemic scleroderma progresses slowly, some have the tendency to remit on their own, do not easily stop or give up treatment.
②Pay attention to physical exercise, reasonable life pattern, avoid emotional stimulation and changes.
③There are no special drugs for the treatment of this disease. Throughout the course of the disease, it is important to actively cooperate with Chinese medicine therapy for the control of the disease.
④Patients with Raynaud’s phenomenon should pay attention to heat preservation and avoid cold stimulation.
⑤Smoking is prohibited.