Pregnancy with scleroderma

Introduction to pregnancy with scleroderma Sclerosis is a disease characterized by localized or diffuse fibrosis or hardening of connective tissue in the skin and internal organs, followed by atrophy. It is a rare chronic connective tissue disease. The incidence of women is 3 to 8 times that of men, and the incidence rate is higher. According to the extent of skin lesions and whether there is systemic damage, it can be divided into two types: localized and systemic (systemicclerosis, SSc). The former is limited to the skin. The latter not only invades the skin, but also affects the esophagus, gastrointestinal tract, lung and kidney. A variety of organs. basic knowledge The proportion of illness: the incidence rate is about 0.002%-0.003% Susceptible population: pregnant women Mode of infection: non-infectious Complications: lupus erythematosus dermatomyositis rheumatoid arthritis Sjogren's syndrome

Pregnancy with scleroderma

(1) Causes of the disease

The cause of the disease is still unclear. At present, most scholars believe that the disease may be an autoimmune disease caused by a chronic infection, which is based on genetics.

Immunology (30%)

In recent years, the disease is considered to be an autoimmune disease. A variety of antibodies can be detected in the serum of patients, such as anti-nuclear antibodies, anti-PSS-RNA antibodies, high gamma globulinemia, circulating immune complexes, etc. .

Genetic factors (25%)

Some patients have a clear family history, and the relatives of the patients have chromosomal abnormalities, which may belong to the dominant X-complex inheritance type.

Infection factor (30%)

Many patients often have a history of acute infection before onset, such as tonsillitis, sinusitis, and pneumonia.

(two) pathogenesis

Current research shows that the pathological changes of SSc are similar to graft-versus-host reactions. Microchimerism may be a promoter. Microchimerism is formed by the migration of a small number of peripheral blood cells between different individuals, such as during pregnancy. Cell microchimerism can occur between maternal and fetal, between fetuses during multiple pregnancies and between donors and recipients during transfusion or organ transplantation. Under certain conditions, such as when the maternal fetus shares certain HLA genes, the fetus origin Cells can cause maternal pathological damage. In 1996, Nelson first proposed the hypothesis that fetal-derived cells in the maternal blood can cause autoimmune diseases, especially SSc. Most women with SSc and some healthy women have blood in their maternal blood. Source cells, but the number of cells in the former is 30 times that of the latter. In 1998, Carol et al found that some SSc female patients had fetal-derived cells in the skin damaged area. In 2002, Johnson et al reported that fetal cells were found in multiple internal organs of SSc patients. .

Infiltration of various inflammatory cells, including CD4, CD8, T lymphocytes, B lymphocytes, mast cells, macrophages, and eosinophils, in the skin, lung, smooth muscle, digestive tract, joint fluid, and liver of patients with SSc Etc., most patients also have the presence of autoantibodies, but at present it is not clear how the pathological damage in patients with SSc occurs.

There are few reports on pregnancy with scleroderma. Johnson (1964) analyzed 337 women with scleroderma before the age of 45 with 36 pregnancies. There was no change in scleroderma during pregnancy, and scleroderma had no significant effect on pregnancy. However, according to Cunninham (1989) experience, symptoms of dysphagia caused by esophageal involvement during pregnancy are aggravated. For example, heart and kidney are accompanied by increased hypertension and pulmonary fibrosis. Renal insufficiency and malignant hypertension are often causes of death in non-pregnant women. Whether it is easy to cause pre-eclampsia during pregnancy and the severity of the disease is still unclear, but it is certain that there is a risk of pre-eclampsia-eclampsia in vascular-kidney lesions before pregnancy. One patient died during pregnancy. Eclampsia.

Pregnancy with scleroderma prevention

Absolutely ban smoking, but you can drink white wine or red wine in a small amount; avoid cold water, cold, cold, tea, coffee and other irritating factors to avoid shrinkage of peripheral blood vessels; keep warm in life, wear early in seasons. Gloves, masks, hats, scarves, earmuffs, cotton shoes, cotton socks, cotton jackets, cotton trousers, hands and feet should be rubbed regularly, appropriate activities, wash your hands with warm water as much as possible to promote peripheral blood circulation.

Pregnancy with scleroderma complications Complications Lupus dermatomy dermatomy rheumatoid arthritis Sjogren's syndrome

Some patients are often complicated by lupus erythematosus, dermatomyositis, rheumatoid arthritis, Sjogren's syndrome or Hashimoto's thyroiditis.

Symptoms of pregnancy with scleroderma Common symptoms Pericarditis, high water swelling, difficulty swallowing, weight loss, angina, arrhythmia, hard spot, hardening, hardening, skin sclerosis

1. Localized scleroderma: The most common plaque-like plaque disease occurs in the abdomen, back, neck, limbs and face. It is initially reddish edematous patch, after several weeks or even months. Enlarged and hardened, it is light yellow or ivory, the surface is dry and smooth, and there is a slight purple-red halo around it. After a few years, it gradually shrinks and thins like a parchment, and even the subcutaneous tissue and muscles shrink and harden. A hardwood-like piece of wood has a sacral phenomenon in the arteries of the extremities (Raynaud phenomenon), and it is prone to refractory ulcers at the site of hardening of the extremities.

2. Systemic sclerosis: Invade the organs of the internal organs, and the joints, lungs, and esophagus are more common. Others such as the cardiovascular system, the gastrointestinal tract, and the central nervous system of the urinary system can be involved.

(1) Bone and joint: Most patients have joint lesions, which are characterized by joint pain and arthritis. Early mild activity is limited, and gradually develops to joint rigidity, contracture deformity, large and small joints are often affected at the same time, and the hand joints are more It is common.

(2) Mucosa: Oral mucosa including tongue, gums, soft palate, throat, etc. can be hardened and atrophy, and can affect the connective tissue and muscles of the eyeball and palpebral conjunctiva, causing narrow pharyngeal gates, incomplete eye closure; may also have oral cavity and The performance of some Sjogren's syndrome such as dry throat.

(3) Lung: extensive pulmonary interstitial fibrosis occurs, coughing, progressive dyspnea, and decreased lung capacity.

(4) esophagus: difficulty swallowing, often accompanied by vomiting, due to reflux esophagitis and sensation of burning behind the sternum.

(5) Cardiovascular: There may be varying degrees of myocarditis, pericarditis or endocarditis with chest tightness, shortness of breath, arrhythmia and angina.

(6) Kidney: Proteinuria, hypertension and azotemia occur due to sclerosing glomerulitis.

(7) Others: In addition to high fever in the early stage of acute onset, there are often low fever, weight loss, and general weakness.

Pregnancy with scleroderma examination

In most patients, elevated erythrocyte sedimentation rate, increased gamma globulin, 1/3 of patients with positive rheumatoid factor test, and 90% or more of patients with antinuclear antibodies and/or anti-nucleoside antibodies.

1. Skin biopsy examination The collagen fibers and the arterioles wall showed pathological changes of specific swelling or fibrosis of the disease.

2. X-ray examination of the two lungs texture thickening, lower esophageal expansion and contraction function is low.

Diagnosis and differentiation of pregnancy with scleroderma

Diagnostic criteria for systemic sclerosis:

1. Having 3 of the following 5 main symptoms can confirm the disease.

(1) Skin symptoms: The initial appearance of the arm, the upper eyelid causes unexplained edema and skin symmetry, diffuse, edematous sclerosis; late skin hardening and finger flexure contracture.

(2) Symptoms of the extremities: Raynaud phenomenon or ulceration or scar formation at the end of the digit.

(3) joint symptoms: multiple joint pain or arthritis.

(4) Chest symptoms: pulmonary fibrosis.

(5) Esophageal symptoms: lower esophageal dilatation and contraction function.

2. Pathological examination has characteristic pathological changes of the disease.

Differential diagnosis

The disease must be differentiated from the following diseases:

1. Hard swollen disease has upper respiratory tract infections, which occur in the neck, shoulders, face and trunk. The limbs are rarely affected. After several months to several years, they can naturally subside. There are special pathological phenomena that can be identified.

2. Eosinophilic fasciitis This disease has no Raynaud's phenomenon, no hypergammaglobulinemia, but there is eosinophilia, skin can be hardened in the limbs, especially the thighs and upper arms show edema-like redness, may have tenderness, Localized sclerosis occurs rapidly, and the pathological inflammatory thickening of the inflammatory infiltrator changes.

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