In most people's concepts, it is believed that diseases will only patronize the elderly, especially cardiovascular and cerebrovascular diseases, which are exclusive to the elderly. As everyone knows, some diseases are more common in young people, and female patients are more favored.
Patient, female, 30 years old, unmarried.
【Main complaint】: Fatigue, nacha with generalized joint pain for 1 month, facial and lower limbs puffiness for 2 weeks.
【Current medical history】: Before 1 month, there was no obvious cause of pain and discomfort in multiple joints of the whole body after bathing, no fever, redness, tenderness, joint deformity, no history of trauma, and no attention and diagnosis and treatment. 2 weeks ago, there was no obvious cause of facial and lower limb puffiness, accompanied by fatigue, poor tolerance, no special treatment, the above symptoms gradually aggravated, palpitations after activity, chest tightness, for further diagnosis and treatment of the Department of Nephrology affiliated to Changzhi Medical College and Ji Hospital.
【Past history】: The history of anemia is about 6-7 years, and the specific degree of anemia is unknown.
【Physical examination】: body temperature 36.3 °C, pulse 97 times / min, breathing 20 times / min, blood pressure 154/110 mmHg, poor mental health, shenqing language, chronic appearance, anemia, facial puffiness, eyelid conjunctiva slightly pale, clear breath sounds of both lungs, no obvious dry and wet rales, strong heart sounds, heart rate 97 times / min, rhythm, no smell and murmur, flat abdomen without tenderness, two lower limbs II degree concave edema, no redness and pain deformity of the joints of the limbs, the lower abdomen and bilateral thigh skin can be seen scattered in the ecchymosis, Needle tip size bleeding point.
Cooperate with urinalysis, blood routine, esolysis, coagulation series, pre-transfusion series, biochemistry and other auxiliary tests.
【Diagnosis and Differential Diagnosis】:
The patient was 1:1280 antinuclear antibody, positive anti-double-stranded DNA antibody, positive panca, positive MPO, and positive antiGBM antibody
Question: Diagnosing systemic lupus erythematosus? ANCA-associated vasculitis? Systemic lupus erythematosus with ANCA-associated vasculitis?
analyse:
(1) Systemic lupus erythematosus is a serious chronic autoimmune disease, often accompanied by positive antinuclear antibodies ANA and anti-double-stranded DNA antibodies. The clinical manifestations of SLE are highly heterogeneous, with about 30% to 60% of patients presenting in the form of lupus nephritis and about 11% of patients presenting in the form of vasculitis.
(2) Lupus nephritis LN is characterized by the deposition of immune complexes in the glomeruli. In contrast, renal-affected anti-neutrophil cytoplasmic antibody ANCA-associated vasculitis is typical of oligoimmune complex neomeric glomerulonephritis. Crescent formation is a hallmark of renal vasculitis, but can also be seen in severe LN.
(3) UP TO 20% of patients with SLE can be monitored, and some patients meet both the diagnostic criteria for SLE and the diagnostic criteria for anti-neutrophil cytoplasmic antibody ANCA-associated vasculitis AAV, which is called [SLE/AAV overlap syndrome].
(4) A document was published in the journal Medicine on a nationwide survey of patients with SLE/AAV overlap syndrome. Findings suggest that SLE/AAV overlap syndrome may occur, but the prevalence is low, the vast majority are female patients, the clinical manifestations are severe, most patients have rapid renal manifestations, and often lung involvement manifested by pulmonary hemorrhage, usually with positive ANA and anti-MPO antibodies.
In summary, the current diagnosis is: acute renal failure, lupus nephropathy, renal hypertension, systemic lupus erythematosus with ANCA-associated vasculitis, lupus-associated hematologic damage, lupus-associated serous effusion.
The next step is to consider improving renal biopsy puncture as soon as possible, clarifying the pathological diagnosis, and assisting in diagnosis and treatment.
After admission, the patient's condition was progressively aggravated, serum creatinine continued to rise, cerebral hemorrhage, secondary epilepsy, sputum embolism appeared in a short period of time, and the condition was critical, and the renal biopsy puncture examination was suspended.
Occipital lobe cerebral hemorrhage
Left pulmonary sputum embolism
In the past, everyone often "talked about wolf discoloration", not only because the disease is harmful, the skin and joint lesions of the light, the heavy involve the internal organs, blood system, nervous system, etc., making people scale all over the body, but also because of the difficulty of treatment and high mortality.
At present, with the development of medicine, there are more and more drugs and technologies that can be applied in the clinic, and lupus is no longer an "incurable disease".
The medical staff of the Department of Nephrology affiliated to Changzhi Medical College and Ji Hospital have grown steadily into an excellent team with sincere unity, hard work and innovation. The patient's condition is critical, the department attaches great importance to it, the director of the department Zhang Wei and the chief physician Zhang Lixia conduct a comprehensive assessment of his condition, and currently need a large dose of hormone shock combined with plasma exchange. Patients are in poor general condition, and high-dose hormonal shocks may have serious complications such as severe infection and gastrointestinal bleeding, which can also be fatal. Plasma exchange is necessary to use a dose of anticoagulant to keep blood flowing smoothly in the tubing, but the use of anticoagulants in patients with cerebral hemorrhage limits the use of anticoagulants. At this time, Liu Jihong, deputy chief physician of the dialysis room, deputy director of the department, deputy chief physician Of the department, deputy chief physician Of Bow Xiaoli, deputy chief physician Wen Wenbin, and deputy chief physician Wen Wenbin worked together to provide advice and suggestions, and the general medical staff overcame many difficulties and fulfilled their responsibilities and actively formulated a treatment plan. On the one hand, while giving patients a large dose of hormones combined with immunoglobulin impact to suppress the immune response, do a good job in various nursing work such as oral care, urethral care, ward disinfection, etc., as far as possible to reduce the chance of infection in patients; on the one hand, citrate plasma exchange, reduce the body's immune response continues to cause serious damage to the system of the whole body and effectively reduce the risk of bleeding.
Absorption phase of cerebral hemorrhage in the occipital lobe
Sputum embolism is lifted
Blood cell changes in the blood routine
Hemoglobin, platelets, erythrocyte sedimentation rate changes
Renal function blood creatinine change map
24-hour urine protein quantification
Complement C3, C4 change chart
The efforts paid off, and with the unremitting efforts of the general staff, the patient's condition turned critical. At present, the patient's vital signs are stable, the indicators tend to improve, and they are in recovery.
What is systemic lupus erythematosus?
Systemic lupus erythematosus is an autoimmune inflammatory connective tissue disease that occurs more often in young women involving multiple organs. The cause of this disease has not been determined so far, and a large number of studies have shown that genetics, endocrine, infection, immune abnormalities and some environmental factors are related to the pathogenesis of this disease.
What are the clinical manifestations of systemic lupus erythematosus?
The disease can be from head to toe, from the outside to the inside, from the skin to the internal organs, involving multi-system multi-organ damage, and the clinical manifestations are diverse. There is systemic fever, fatigue, anorexia, weight loss, skin and mucous membranes can see butterfly erythema, discoid erythema, hair loss, mouth ulcers, photoallergic, reticular blue spots, Raynaud's phenomenon, etc., skeletal muscles are manifested as arthralgia, arthritis, myalgia, muscle weakness, and in severe cases, pericarditis, pleurisy, nephrotic syndrome, renal failure, mental abnormalities, convulsions, cerebrovascular accidents, etc., which are seriously life-threatening.
How to identify and treat it as early as possible?
For unexplained fever, fatigue, hair loss, general malaise, weight loss, facial erythema, repeated mouth ulcers, photosensitivity, joint swelling and pain, and some only manifest as hematuria, proteinuria, anemia, white blood cells, thrombocytopenia, especially women of childbearing age, once they have the above symptoms, they need to come to the hospital as soon as possible for treatment, and further improve the immunological examinations such as antinuclear antibodies, anti-double-stranded DNA antibodies, anti-Sm antibodies, and complements to confirm the diagnosis.
How to properly face systemic lupus erythematosus?
At present, the disease cannot be cured, but there are enough drugs and means to alleviate the disease for a long time, and most patients can live a normal life. Do not trust false advertisements and ancestral secret recipes. Timely go to the local regular hospital for diagnosis and treatment, take the medicine on time, review it regularly, and gradually reduce the amount of drugs. The induction and recurrence of this disease have a certain relationship with mental factors, pay attention to maintaining a good emotional and mental state, and face the disease optimistically.
(Source: Heji Hospital Affiliated to Changzhi Medical College)
(Editor-in-Charge: Zhang Wenwei)