Systemic lupus erythematosus

Systemic lupus erythematosus

Summary

SLE displays multi system chronic systemic one's own immune disease which damages symptom, SLE patient serum has many kinds of one's own antibodies specializing in resisting the nuclear antibody, relieve and acute and break out and replace as the characteristic with the condition. The good hair, to the reproduction age women 20- 40 years old, the ill rate of our country is 1/1000. The precise cause of disease has not been bright, relate to many kinds of factors:

1.Heredity: The morbidity of SLE is high of some patient relatives.

2.Environmental factor: Sunlight, ultraviolet ray, medicine, microbial pathogen,etc..

3.Estrogen: There are much women before the onset is in climacteric, the man: Female =1:9.


Pathology

Main pathology is changed into inflammation reacts and the blood vessel is unusual. The immune complex of medium and small blood vessel is deposited, the antibody attacks inflammation and necrosis of tube wall directly, continue the hair and organize the ischemic and function obstacle. The characteristic change of the damaged organ: (1)Small purple body of wood of Jiangsu; (2)" onion peel kind " Pathological change.

WHO divide the intersection of glomerulus and pathological change, lupus of nephritis into six type:

Normal or slight pathological change type (I type) ,It is membrane pathological change type (II type) ,Cooking stove hyperplasia type of the office (III type) ,Hyperplasia filling the air type (IV type) ,Membrane pathological change type (V type) ,Hardening type of glomerulus (VI type) .


Clinical manifestation

1,Whole body symptom: Generating heat, tired, weight decline,etc.;

2,The mucous membrane of skin: 80% of the patients present rash in the course of disease; Erythema of shape of butterfly of cheek department, have characteristic nature the most; Discal erythema; Point palm or first week erythema.

The special skin type correlated to SLE:

A, sub-acute skin type lupus erythematosus SCLE: Rash is extensive, lie in the exposed position, the simple table of pathological change, present symmetry, can form blister or great blister sometimes, heal and does not leave cicatrix

B, deep lipoprotein membrane inflammation type: Relatively rare, involve the deep layer of dermis and lower fat floor of the cover, does not involve the epidermis, is shown as tubercle under the skin.

3,Serous coat inflammation: The thorax accumulates the liquid, pericardium and accumulates the liquid.

4,Muscle skeleton: Arthralgia, red swelling, femur head necrosis, myalgia, myositis.

5,Nephritis LN of the lupus: About 75% of the patients have clinical manifestation of LN, urine unusual, nitrogen blood disease, kidney hypertension, uremia. Shown as chronic nephritis type, nephropathy and syndrome type, it is radical nephritis type to be even.

6,Cardiovascular: Pericarditis, the cardiac muscle is damaged, the heart valve disease, the vascular pathological change around, uncomfortable in the district before there can be shortness of breath, heart, arrhythmia, in heart failure.

7,Lung

35% of the patients have in one pair of sides a small amount of thoraxes accumulate the liquid, lupus pneumonia, quality pathological change among the lung, artery high pressure of lung.

9,Digestive system

Digestive system symptom and the enteric wall and vascular inflammation of the mesenterium are relevant, about 30% of the patients have losing the appetite, suffering from abdominal pain, vomiting, diarrhoea, ascites,etc., about 40% of the patient serum transaminase is raised, minority erupt acute abdomen simultaneously, such as pancreatitis, intestines necrosis, intestinal obstruction.

10,Blood system

Anaemia: See in 60% of the activities period SLE, 10% is AIHA, Coombs tests the masculine gender;

The leucocyte is reduced: 40% of the patients, it may be positive to resist the neutral cells afterbirth thick liquid antibody;

The blood platelet is reduced: 20% of the patients, resist the blood platelet antibody positively;

The light - intermediate enlargement of lymph nodes: 20% of the patients, pathology shows the reactive hyperplasia of lymphoid tissue, minority are necrosis lymphnoditis;

Splenomegaly: 15% of the patients.

11,The eyes: About 15% of the patients have vascular inflammation of retina; Vascular inflammation can involve the optic nerve.


Laboratory and other inspections

(1) Resist the nuclear antibody table

1.Resist the nuclear antibody ANA

ANA screens the main test of desmosis, see to almost all the SLE patients, it is specific and low.

2. Resist dsDNA antibody

Diagnose one of the mark antibodies of SLE, appear in the activity period of SLE more.

3. ENA antibody

( 1) Resist Sm antibody: Diagnose one of the mark antibodies of SLE. The specificity is 99%, 25% of sensitiveness. It does not represent disease activity that positive.

( 2) Resist RNP antibody: Positive rate is 40%.

( 3) Resist SSA (Ro) Antibody: Often amalgamate mother of dry syndrome and neonatal lupus erythematosus in SCLE, SLE.

( 4) Resist SSB (La) Antibody: Clinical meaning its and it resists SSA to be same in antibody.

( 5) Resist rRNP antibody: It explains condition activity, have the damages of NP lupus or other important viscera to brief on that positive.

Complement

The commonly used ones include total alexinic ( CH50) , the detection of C3, C4. C3 drops one of the indexes that is SLE activity, C4 is low besides expressing SLE activity, it still may be that SLE is apt to be perceptual (C4 is scarce) Manifestation.

First, lupus tape are tested

Measure with the intersection of immunity and fluorogenic law dermis and the intersection of epidermis and intersection of skin have immunoglobulin (Ig) (Ig) Deposit and take, the positive rate of SLE is about 50%, take the test to represent SLE activity in lupus.

Second, living kidney examine pathology

Estimate valuably about the diagnosis, treatment and prognosis of lupus nephritis, X-ray and image learn to check.


SLE
Criteria for classification (U.S.A.'s rheumatism society revised in 1997)

1.Erythema of cheekbone department, 2. Discal erythema, 3. Only allergic, 4. Ulcer of oral cavity, 5. Arthritis, 6. Serous coat inflammation, 7. Kidney pathological change, 8. Nervous system pathological change, 9. The blood system is unusual, 10. Immunology is unusual (the difference of the diagnostic standard was in and 1982 it is changed into APL positively that this lieutenant general's lupus cell was positive) ,11.It is positive to resist the nuclear antibody.


SLE
Judgement of the condition

Active daily SLE disease activity index (SLEDAI) of disease Carry on the assessment


Severity of disease

Position and intensity of the organ according to being given much trouble, main internal organs such as companion's heart, brain, kidney,etc. are given much trouble, take place or AIHA blood platelet reduce companion bleed, indicate pathological change to be serious inclined to, infection, hypertension, diabetes,etc. amalgamate disease to make the condition aggravate.


Differential diagnosis

Need it with various dermatitis, epileptics, psychosis, specially taking place blood platelet reducing purpura, primary glomerulus nephritis, medicine lupus, rheumatoid and arthritis and other desmosis,etc. are distinguished


Treat

Diagnose in early days, treat in early days, the individual treats.

Treat the principle: Disease activity and patient with heavy condition, controlled by the strong medicine, after the condition is relieved, accept the keeping treatment. Deal with the case difficult to control, rescue SLE critical disease. Deal with or prevent and cure the medicine side effect.

1.Generally treat

Psychotherapy, make the patient set up optimistic mood to disease, acute activity one should stay in bed, avoid the risk factor and stimulate, the medicine, the sunshine is tanned by the sun and shone with the ultraviolet ray, pay attention to practising contraception, prevent and cure and infect.

2.Glucocorticoid GC

Walk according to the condition and response to hormone the individual uses, to the not very serious case, splash the Buddhist nun loose daily 1mg/kg, clothes once from morning, decrement, reduce 10% every 1- 2 weeks, reduce to daily 0.5mg/kg 4- 8 weeks later gradually, decrement should be slower, the keeping amount again. Hormone assaults the treatment: Used in acute breaking out critical SLE, first splashes nylon l000mg/d, uses together for 3 days, then splash Buddhist nun's loose treatment.

3.Immune inhibitor

More serious SLE, employs heavy dose of hormone to unite immune inhibitor, controls SLE activity better, reduce SLE and break out, reduce hormone and should monitor the side effect of immune inhibitor.

( 1) Ring phosphorus amide CTX

CTX assaults the treatment, the dosage 10- 16mg/ kg each time, once every 2- 4 weeks, after 6 times, change into once every 3 months, it takes one year after the activity is resting;

Side effect: React gastrointestinal tract, inhibit,etc. depilating, liver damage and bone marrow, at blood leucocyte 3* 109 /at the time of L, delay using.

( 2) Sulphur thiazole purine

Curative effect is not so good as CTX, suitable for the medium-sized serious case, the internal organs function worsens slowly person, dosage daily oral 2mg/ kg.

( 3) The ring spore is plain

Heavy dose of hormone unites immune inhibitor to use for 4- 12 weeks, the condition is not improved yet, should add and use the ring spore plainly, daily 5mg/ kg, divides into clothes 2 times, decrement, keep treating 3 months later gradually, notice the liver, kidney damaging.

( 4) Does Mai take an exam of second ester MMF of phenol

But and hormone, or the application at the same time of other immune inhibitors.

( 5) Hydroxy chloroquine

Effective to rash, arthralgia and light patient, have obeyed and may have influenced eyesight for a long time.

( 6) Rattan total glucoside of Thunder God

There is certain curative effect, the bad reaction is relatively big, such as the toxicity to the gonad.

4.Heavy dose of immunoglobulin (Ig)

It suitable for IVIG intravenous injection some serious but it last the weak extremely physique or ( Sum) Erupt the infeted simultaneously seriously, daily 0.4g/kg, the vein is instilled, use together for 3- 5 days.


Medicine healing solution

Light: With leather a damage and (or) Arthralgia for main fact, can select the intersection of hydroxy and the intersection of chloroquine and (chloroquine) for use ,Complement and resist inflammation medicine with the body of non- steroid. There is not effect that take hormone as soon as possible, splash the Buddhist nun loose 0.5mg/kg every day.

So so: There are generating heat, cover decreasing, arthralgia and serous coat inflammation, and the person who has slight proteinuria, it is loose to splash the Buddhist nun, daily 0.5mg- 1mg/kg.

Heavy-duty: Accompany internal organs such as the heart, brain, kidney,etc. to be given much trouble, while dissolving the poor or blood platelet to reduce companions to bleed and is inclined to, splash the intersection of Buddhist nun and loose daily 1mg/ kg or first splash nylon and bludgon the treatment, the immune inhibitor treatment meanwhile into.

Relieve one: The clothes splashes the Buddhist nun loose 7.5mg in the morning of every day.


SLE
And gestation

SLE can possess following terms person safe gestation:

There are no central nervous system, kidney or heart to damage, more than of inactive immune inhibitor March, splash Buddhist nun, keep while being loose while being low dose of only, condition relieve more than half a year. Monitor closely. Only if relieve a SLE apt miscarrying, premature delivery or stillborn fetus, gestation can bring out SLE activity, it has especially been early for and postpartum 6 weeks in gestation. There is medical history of habitual abortion or resist the positive one of phosphatide antibody, should take aspirin of low dosage (50mg/d) at the time of gestation . Hormone is deactivated while passing the placenta ' But if you can't fill by exception by rice in) ,Can not be harmful to foetus, can treat hormone according to the need of condition at the time of gestation and in postpartum one month. Avoid nursing postpartum.


Prognosis

Survival rate: About 85% 5 years, about 68% about 75%, 20 years of 10.

There is prognostic difference of the following one: Skin anhydride of blood has already risen; Hypertension; It is not complete that the cardiac muscle damages companion's heart function; Serious NP lupus.

The person who dies of SLE own pathological change accounts for half. The most common one is that renal failure, brain are damaged and in heart failure. Die of SIE person who erupts disease simultaneously and also account for half, mainly infects.


Systematicness Lupus erythematosus
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