Systemic lupus erythematosus (SLE) is an autoimmune disease of unknown etiology, and multisystem damage is more common. The prevalence of SLE in China is about 30-70/100,000, and about 90% of the patients are women, mostly women of childbearing age 20-40. Li Tianwang, Department of Rheumatology and Immunology, Guangdong Second People’s Hospital
The diagnosis of SLE has always been a difficult problem for doctors, with misdiagnosis occurring from time to time and causing many treatment errors. So, what makes the misdiagnosis rate of this disease so high? Li Tianwang, director of the Department of Rheumatology and Immunology of the Second People’s Hospital of Guangdong Province, introduced that SLE is an autoimmune disease involving multiple systems throughout the body, with no fixed pattern of clinical manifestations, and the disease is prolonged and recurrent, with periods of remission of varying length. “SLE with typical clinical symptoms is not difficult to diagnose, but it is very easy to miss and misdiagnose patients with atypical early symptoms, or those limited to single or few organ damage and those without rash manifestations.”
The cause of the disease is unknown, the lesions are diverse
Li Tianwang pointed out that the exact pathogenesis of SLE is still unclear, but its basic pathological change is vasculitis formed by antigen and antibody immune response. Therefore, all tissues with blood vessels can be involved, resulting in tissue damage and corresponding organ dysfunction, such as skin, joints, muscles, bones, heart, lungs, liver, spleen, kidney, brain, spinal cord, eyes, ears, mouth, nose, blood system and peripheral nerves, and then produce the corresponding clinical manifestations. However, the onset of the disease varies from person to person, and the number of organs involved, the sequence of onset and the degree of damage also vary, so the clinical manifestations are diverse.
In general, patients with SLE may have symptoms such as fever, fatigue, loss of appetite, general malaise, joint swelling and pain, muscle pain and weight loss in the early stage of the disease. Some patients may have specific symptoms, such as hair loss or facial erythema. Some patients may have multiple symptoms, such as high fever, arthralgia, erythema of the fingertips or episodes of bruising when the hands and feet are cold; or recurrent oral ulcers, swollen superficial lymph nodes, or menstrual bleeding; or purpura of the skin, anemia, decreased white blood cells and platelets; or psychiatric symptoms: headache, hallucinations, hallucinations, or a state of rigidity; or persistent diarrhea, vomiting; or elevated transaminases, jaundice; or pleural fluid, pericardial effusion, palpitations. Pleural fluid, pericardial effusion, palpitation and shortness of breath, inability to lie down, etc.
Because the above symptoms can appear in a variety of non-rheumatic immune diseases, it makes the diagnosis of the disease more difficult, and is often misdiagnosed as common nephritis, or as tuberculous pleurisy due to pleural fluid; or as aplastic anemia or even leukemia due to anemia, leukocytopenia, or thrombocytopenia; and jaundice is easily misdiagnosed as viral hepatitis; fever and swollen lymph nodes are misdiagnosed as lymphoma, or neurological symptoms are misdiagnosed as psychosis, etc. The symptoms of fever and swollen lymph nodes are misdiagnosed as lymphoma or neurological symptoms as psychosis.
Pawned off as tuberculous pleurisy
Case: Li Bo, a 53-year-old farmer from Lianzhou, was hospitalized locally for chest and abdominal pain in August 2014 and went to work in Guangzhou after getting better. In September of that year, he developed fever and dry cough with no obvious cause, facial and limb edema and joint pain, and after treatment at a clinic near the construction site, the upper limbs and facial edema disappeared, but the lower limbs were still edematous.
One month later, Li Bo’s symptoms worsened and he went to the local hospital again, where he was diagnosed with “tuberculous pleurisy” after examination of a small amount of bilateral pleural effusion and a chest X-ray showing thickening of the right interlobular pleura, In November 2014, Li was transferred to the Department of Rheumatology and Immunology of Guangdong Province Second Medical Doctor for treatment, and was finally diagnosed with pleurisy caused by systemic lupus erythematosus, and his condition was quickly controlled after adjusting the treatment plan.
Li Tianwang introduced that the lung is also a common site of SLE involvement, which can lead to a variety of lung diseases. For example, lung infection, pleurisy, acute lupus pneumonia, alveolar hemorrhage, upper respiratory tract dysfunction and so on. Among them, SLE pleurisy is often misdiagnosed as tuberculous pleurisy.
Li Tianwang said that many doctors are more aware of and alert to lupus nephritis, while ignoring the damage to the lungs and pleura. Especially for some middle-aged and elderly male patients, with imaging and some respiratory symptoms, the first consideration is tuberculosis or tumor. However, if their treatment is not effective with anti-inflammatory and anti-tuberculosis therapy, but effective with glucocorticosteroid therapy, they should think of autoimmune system problems and have a complete immune test.
Treat as common nephritis
Case: Miss Liu, 21, who was engaged in the beauty industry, developed facial erythema, hair loss, swelling and pain in the knuckles of both hands and swelling in the lower extremities three years ago, and proteinuria was found. After the standard immunomodulation treatment, the disease was once stable, but then Ms. Liu stopped the medication on her own and only took traditional Chinese medicine treatment. A few months later, Ms. Liu’s condition recurred, with high generalized swelling, oliguria, shortness of breath, large accumulation of fluid in the chest, abdomen and pericardium, heart, lung and kidney failure and combined with fungal infection in the lungs, and finally her life could not be saved despite active resuscitation and spending hundreds of thousands of yuan.
For SLE, many people first think of skin damage such as facial erythema, but do not know much about its relationship with kidney, thus delaying the best time for treatment. Why does SLE affect the kidneys? Li Tianwang said the mechanism is very complicated, mainly related to the deposition of autoantigen-antibody complexes in the kidney tissue, which causes a series of immune damage reactions.
According to the relevant literature, about 27.9%-70% of SLE patients have different degrees of clinically visible kidney damage at the time of diagnosis. If a renal biopsy is performed, pathological changes in the kidney tissue can be found in almost all patients. Patients with lupus nephritis may present clinically with acute, acute, insidious or chronic nephritis or nephrotic syndrome. In the early stage, the symptoms are mostly asymptomatic urinary abnormalities, and in the progressive stage, the symptoms are increased foam in the urine with edema, hypertension and hyperlipidemia; in a few patients, the disease starts rapidly and the renal function deteriorates within a short time or even acute renal failure occurs. If the active lesions are not effectively controlled and the disease persists, some patients may gradually progress to chronic renal insufficiency or even uremia, which is also a common cause of death in lupus patients.
Mental illness
Case: Mr. Wang’s 20-year-old daughter, who usually has a good personality, suddenly cursed and quarreled for no reason for some time, and then gradually developed a low fever. After a period of hormone treatment, his daughter’s condition improved for a while, but after stopping the hormone, the symptoms flared up again, and after 3 months of treatment, she did not improve, but developed a high fever and severely reduced blood white blood cells, and the hospital once issued a notice of critical condition. Mr. Wang is very angry, said spent more than 100,000, daughter was treated like this, demanding that the hospital take responsibility. Later, the hospital held a consultation, and only then the rheumatology experts suggested the possibility of SLE and lupus encephalopathy, and after timely adjustment of treatment direction, the condition was quickly controlled.
Li Tianwang introduced that 20% of SLE patients have neurological lesions, “the brain, spinal cord and peripheral nerves can be damaged. Among them, the brain is mainly involved, and clinically there are mental disorders, epilepsy, hemiplegia, bleeding, etc.” Spinal cord lesions and peripheral neuropathy occur in a small number of patients. Therefore, before diagnosing lupus encephalopathy, it is important to exclude mental abnormalities caused by high doses of hormones, or uremia, electrolyte disorders, or diabetic ketoacidosis, as well as headaches or mental symptoms caused by hypertension.
Lupus encephalopathy mainly occurs in the active stage of lupus erythematosus, and a series of psychiatric and neurological symptoms appear because the lesions involve the central nervous system. In addition, patients may also have symptoms such as emotional agitation, depression, irregular sleep, nightmares or nausea and vomiting, or near-madness, or epileptic-like seizures during infections in the brain or medium or high doses of hormone therapy, which need to be distinguished from lupus erythematosus encephalopathy in clinical practice.
The occurrence of lupus erythematosus encephalopathy may be due to the precipitation of immune complexes in small blood vessels, forming small embolisms, causing ischemia and hypoxia in the central nervous system, and also causing damage to the peripheral nervous system. The neurological lesions can be focal or diffuse. The most common symptoms of neurological damage are epilepsy, followed by cerebrovascular disease, increased intracranial pressure, and aseptic meningitis.
Habitual abortion
The case: Qingyuan’s Ms. Shi, 6 months pregnant in 2013, the fetal development stopped, resulting in miscarriage, at that time she did not look for further reasons. 2014, Ms. Shi pregnant again, when the eighth week of pregnancy, the fetus again stopped developing and miscarriage, then suspected of “habitual miscarriage”, after detailed examination, only to uncover the real “killer” – the The real “killer” – SLE – was discovered after detailed examination.
Li Tianwang said that about 20-40% of SLE patients’ disease worsens during pregnancy. Studies have found that when SLE is active, once a patient becomes pregnant, the condition may not only worsen, proteinuria, hypertension and edema may occur, but also miscarriage, premature birth and fetal failure may occur.
According to statistics, the fetal mortality rate of these patients is 2-3 times higher than normal; 60% of the delivered fetuses are below normal weight. This is because when the mother is in the active stage of SLE, the immune complexes are deposited in the basement membrane of the placenta trophoblast layer, resulting in poor blood supply to the placenta and thus affecting the blood circulation of the fetus, leading to fetal growth retardation or even death.
In the past, SLE was considered a contraindication to pregnancy, but with the development of medical science, many SLE patients can now have a successful pregnancy and deliver a healthy fetus, but the following conditions must be met before pregnancy can be considered:
1. No significant organ involvement;
2. The disease remains in remission for at least six months, preferably more than one year, after the hormone dose has been reduced to the equivalent of prednisone ≤ 10 mg/dose;
3. The use of potentially teratogenic immunosuppressive drugs has been discontinued for at least 6 months.