overlap syndrome
Introduction
Introduction to overlap syndrome Overlap syndrome (Overlapsyndrome) refers to the overlap of two or more connective tissue diseases, also known as overlapping connective tissue disease. This overlap can occur at the same time. A good patient can meet the diagnosis of two or more connective tissue diseases at the same time; another connective tissue disease can occur in different periods; or there is a certain connective tissue disease. The transition is transformed into another CTD. This transition can be carried out in succession or at intervals. OS usually occurs in 6 diffuse CTDs, ie overlaps of SLE, RA, DM/PM, PSS, nodular polyarteritis (PN) and rheumatic fever (RF), or 6 CTDs and related diseases such as white Plugging disease, Sjogren's syndrome, and panniculitis overlap, and it can overlap with other autoimmune diseases such as chronic thyroid sinusitis and autoimmune hemolytic anemia. basic knowledge The proportion of illness: 0.005% Susceptible people: no specific population Mode of infection: non-infectious complication:
Cause
Cause of overlap syndrome
(1) Causes of the disease
Western medicine: The etiology of overlap syndrome is unclear, but it is mainly related to immune dysfunction, environmental factors and genetic background.
Traditional Chinese medicine: abnormal congenital endowment, long-term illness, lung deficiency, spleen and kidney yang deficiency, each aggravated by feeling exogenous evil, long-term sputum resistance. Clinical can be divided into phlegm and blood stasis, phlegm and heat, , Five syndromes of lung and spleen deficiency, lung and kidney qi deficiency syndrome differentiation and treatment.
(two) pathogenesis
The pathogenesis is also clear, may be a process of collagen disease conversion to another collagen disease.
Prevention
Overlap syndrome prevention
Prevention of connective tissue diseases is the key.
Complication
Overlapping syndrome complications Complication
The main complications of this disease:
1. Cardiovascular complications;
2, central nervous system damage;
3, kidney failure;
4, severe cases can die.
Symptom
Symptoms of overlap syndrome Common symptoms Powerless joint deformity Subcutaneous nodules Skin sclerosis Myalgia Difficulty Immune hemolytic discoid erythema Hemolytic anemia Leukopenia
Although overlapping syndrome can occur in overlapping combinations of all connective tissue diseases and their related diseases, the cases actually seen are mainly SLE, overlap between PM/DM and PSS.
1. SLE and PSS overlap often appear as SLE at the beginning, and later skin hardening, dysphagia and pulmonary fibrosis, the incidence of facial erythema is lower than that of simple SLE, the incidence of Raynaud's phenomenon is high, and the anti-dsDNA titer is low. LE cell positive rate is low, ANA is high-priced, high positive rate, the composition is anti-NDA antibody, and the fluorescent karyotype is spotted.
2. SLE overlaps with PM in addition to SLE performance, there are proximal muscle weakness, myalgia and tenderness, atrophy and induration, high serum ANA positive rate, low LE cell detection rate, low complementemia, high gamma globulinemia, Serum sarcoplasmic enzymes such as CPK, LDH and aldolase increased, and 24-hour urinary creatine excretion increased.
3. SLE and RA overlap in addition to SLE symptoms of arthritis, joint deformity and rheumatoid nodules, serum RF showed a high rate of high positive rate.
4. SLE overlaps with PN When SLE overlaps with PN, in addition to SLE performance, there are subcutaneous nodules and abdominal pain along the blood vessels. Kidney damage is more serious than single SLE. Pulmonary symptoms and central nervous system involvement are common. Acidic cells increase, gamma globulin is high, but LE cell positive rate is low.
5. Patients with overlapping PSS and PM/DM have proximal muscle weakness, myalgia, joint pain, slowing of esophageal movement and pulmonary fibrosis. Scleroderma changes are often limited to limbs, telangiectasia and acral ulcers are rare. Serum Ku, PM-Scl-70 and other U2RNP antibody positive characteristics.
6. Other forms of overlap can be changed. Usually, the most common overlap of CTD and its related diseases is Sjogren's syndrome. Others are Behcet's disease, lipitis and Hashimoto's thyroiditis.
Examine
Overlap syndrome check
1.SLE and PSS typical OCTD:
(1) SLE and PSS typical OCTD:
1 gamma globulin increased, immunoglobulin increased,
2LE cells have a low positive rate.
3ANA positive is high titer,
4 anti-DNA antibody positive rate is low and low titer,
5 Unlike simple SLE, the fluorescent antibody type is spotted.
(2) MCTD:
1 anti-RNP antibody positive high titer (> 1: 1024),
2 anti-Sm antibody negative,
3ANA positive, spotted,
4 immunopathology showed that the skin epidermal spine cell nuclear chromosome was positive.
2. SLE and PM (DM) OCTD:
1 uric acid and serum enzyme activities (GOT, GPT, LDH, CK, ALD) were significantly increased;
2ANA positive and LE cells positively high;
3 EMG abnormalities.
3. OCTD of SLE and RA: It has the following characteristics:
1LE cells are mostly positive;
2ANA positive, mostly spotted;
3 serum RF was mostly positive.
4. SLE and PN OCTD:
1 In addition to the general immunological abnormalities of OCTD, the reduction of leukopenia is not obvious, often accompanied by an increase in eosinophils;
Part 2 is still positive for hepatitis B surface antigen (HBsAg).
5. SLE and TTP OCTD:
1 In addition to the general immunological changes of SLE, there may be positive cell positive anemia, and platelets are significantly reduced;
2 anti-human globulin test was positive.
Diagnosis
Diagnosis of overlap syndrome
diagnosis
When the same patient has two or more CTDs and their related diseases at the same time or in succession and meets their respective diagnostic criteria, they can be diagnosed as overlap syndrome. The diagnosis should indicate which two types of CTD overlap or a certain type. Overlap syndrome.
The classification of overlap syndrome is not uniform, and most of them are classified into four types, namely, type I, II, and III, in the classification of Fujiwara, domestic Qin, etc. [10] combined with the clinical and laboratory characteristics of 122 cases of OS. Da Tengzhen's classification is consistent. Type IV refers to overlap between CTD-related diseases or CTD-related diseases and other autoimmune diseases, such as SS+ Behcet's disease, SS+ autoimmune hemolytic anemia, etc. Can IV be used as overlapping synthesis The levy has not yet been recognized.
Diagnose based on
1.SLE and PSS typical OCTD:
(1) SLE and PSS typical OCTD:
1 There is a typical SLE, but the incidence of erythema on the cheeks is less, Renault phenomenon and kidney involvement are more, followed by PSS characteristic manifestations such as skin sclerosis, pigmentation, difficulty in swallowing and mouth opening.
2 gamma globulin increased, immunoglobulin increased,
3LE cells have a low positive rate.
4ANA positive is high titer,
5 anti-DNA antibody positive rate is low and low titer,
6 Unlike simple SLE, the fluorescent antibody type is spotted.
(2) MCTD: The clinical manifestations are a mixture of clinical symptoms of SLE, PSS, and PM, but there are conditions for the diagnosis of a certain disease alone. Clinical features: milder disease, less visceral damage (especially kidney); good response to corticosteroid therapy; The prognosis is good; laboratory tests must have the following four conditions:
1 anti-RNP antibody positive high titer (> 1: 1024);
2 anti-Sm antibody negative;
3ANA positive, spotted;
4 immunopathology showed that the skin epidermal spine cell nuclear chromosome was positive.
2. The OCTD of SLE and PM (DM) is different from pure PM (DM) and has the following characteristics:
1 women account for the vast majority;
2 most of the age is under 40 years old;
3SLE is associated with low muscle strength, bone atrophy and muscle induration in the proximal muscle;
4 Both the cheek erythema of SLE and the erythema erythema of DM, sometimes accompanied by the discoid erythema of LE and the Gottron sign of the knuckle extension of DM;
The incidence of more than 5 organs is high and more serious.
6 laboratory examination, creatinine and serum enzyme activities (GOT, GPT, LDH, CK, ALD) were significantly increased; ANA positive and LE cells were positively high; electromyogram abnormalities, neurological control disorders and primary myopathy See the aspect.
3. The OCTD of SLE and RA has the following characteristics:
1SLE showed obvious rheumatoid nodules, arthritis, joint deformity and rigidity,
2 more organ damage, especially obvious kidney damage,
3 laboratory tests showed that LE cells were mostly positive; ANA was positive, mostly spotted; serum RF was mostly positive.
4. SLE and PN OCTD SLE with peripheral neuritis, subcutaneous nodules, lung symptoms and abdominal pain; laboratory tests in addition to general immunological abnormalities of OCTD, white blood cell reduction is not obvious, often accompanied by eosinophils Increased, some have hepatitis B surface antigen (HBsAg) positive.
5. SLE and TTP OCT SLE with obvious purpura, hemolytic anemia and central nervous system symptoms, laboratory tests have reduced general immunological changes in SLE, there may be positive cells positive pigment anemia, thrombocytopenia; anti-human ball The protein test was positive.
Differential diagnosis
1. Mixed connective tissue disease literature in the literature of the genus Dentin also attributed MCTD to type II overlapping connective tissue disease, and it is now considered that MCTD should be considered as an independent disease because patients with overlap syndrome do not have high titers of anti-nRNP antibodies, and In the case of scleroderma similar to MCTD, the overlap syndrome of SLE and polymyositis generally has a poor prognosis.
2. Undifferentiated connective tissue disease (UCTD) The disease refers to some clinical manifestations of patients with connective tissue disease, but a fashion can not be diagnosed as which kind of connective tissue disease, often through a period of clinical observation and regular A laboratory check-up is required to obtain a diagnosis.
