Acute myocardial infarction was admitted to the hospital, it turned out to be the fault of this rheumatism!

It is only for medical professionals to read for reference.
The condition is far from being as simple as it seems! Elderly women, who have had multiple traditional cardiovascular risk factors in the past, were admitted to the hospital because of chest pain and right upper limb pain.
After admission, they developed respiratory failure and changed the color of their fingers.
Can you think of the final diagnosis? Case introduction The patient is a 69-year-old female with a history of "hypertension, hyperlipidemia, type 2 diabetes, and peripheral vascular disease"
.

This time he was admitted to the hospital due to recent episodes of chest pain and right upper limb pain
.

The patient developed cough and sputum 1 week ago
.

On admission to the hospital, his body temperature was 38.
1°C, his right upper limb was slightly pulsating on palpation, his lungs were auscultated with reduced breath sounds, and his heart was normal on auscultation
.

Laboratory tests revealed mild anemia, white blood cell count, procalcitonin, troponin, and blood sugar were significantly increased
.

The dual-power scan of the upper limb veins showed that the axillary and brachial veins were partially occluded, accompanied by thrombosis involving the right vein and cephalic vein (Figure 1)
.

Figure 1.
Dual-power scan of the upper extremity veins on chest CT showed multiple patchy shadows, suspected pneumonia, and no evidence of pulmonary embolism was found
.

Admission ECG: ST segment depression in leads V1-V3, ST segment elevation in leads II, III, aVF, and V7-V9, consistent with acute myocardial infarction (Figure 2)
.

Figure 2.
The electrocardiogram of admission and the patient was rushed to the cardiac catheterization laboratory.
The angiography revealed that the opening of the left circumflex branch was completely occluded.
For this reason, a drug-eluting stent was implanted (Figure 3)
.

Figure 3.
Coronary angiography patients begin to receive appropriate antiplatelet therapy and anticoagulant therapy, while intravenous antibiotics are used to treat pneumonia
.

Later, the patient suffered from acute respiratory failure during hospitalization
.

Echocardiography revealed new ischemic cardiomyopathy, with an ejection fraction of 25%-30%, and the entire inferior lateral wall and inferior wall were retarded
.

One week after admission, the patient developed acute and painless color changes in the left middle finger, which appeared to be black
.

The patient gradually developed dry gangrene of the fingers and extended to the base of the middle finger
.

There is no related cellulitis
.

Due to the underlying hypercoagulable state, this is considered to be an embolism during multiple thrombosis
.

Therefore, the patient further improved the immunological examination: positive antinuclear antibody (1:160, spot type), elevated anti-β2 glycoprotein antibody (>150, reference range <20SAU), anticardiolipin antibody (>120, reference range < 14GPL), anti-phosphatidylserine antibody (>100, reference range <10U/ml), positive for lupus anticoagulant
.

Based on the above information, have you found the real culprit? The physician in charge finally made the inference of catastrophic antiphospholipid syndrome (CAPS)
.

The patient started receiving glucocorticoids and 5 cycles of plasma exchange treatment
.

Before discharge, the patient was treated with bridging anticoagulant drugs and oral warfarin anticoagulant therapy
.

Re-examination of various indicators improved compared with the previous, and the anti-β2 glycoprotein antibody, anti-cardiolipin antibody, and lupus anticoagulant were still positive
.

One month later, the patient returned to the clinic again.
The examination found that the right internal jugular vein was acutely occluded.
After the INR was tested, he was given a larger dose of warfarin and followed up closely
.

Case summary In this case, the patient has thrombosis in multiple organs, and then consider the diagnosis of CAPS
.

However, patients also have multiple traditional cardiovascular risk factors, which puts patients at higher risk of thrombosis
.

After the patient's acute thrombotic event was resolved, the patient was discharged from the hospital to continue anticoagulation therapy, but vascular occlusion occurred again outside the hospital
.

Let's understand the relevant knowledge of antiphospholipid syndrome~ Antiphospholipid syndrome (APS) is a rare autoimmune disease, manifested by hypercoagulable state and arteriovenous thrombosis, with or without pregnancy complications
.

The clinical manifestations of this disease are complex and diverse, often involving multiple organs
.

The most serious form of APS is CAPS, with an incidence of less than 1%, and thrombosis often occurs in multiple organs
.

Its incidence is related to anti-β2 glycoprotein antibody, lupus anticoagulant, and anticardiolipin antibody
.

The first reported case of CAPS dates back to 1984; however, it was not until 1992 that Dr.
Ronald Asherson defined it as a generalized coagulation disorder associated with APL
.

Investigation and research have found that in most cases, there are often some triggers that lead to acute thrombotic events, such as infection, cancer and chemotherapy, surgery, and even pregnancy are common triggers
.

In 2019, a retrospective study conducted in China included 13 CAPS patients.
The study found that the prognosis of patients with CAPS disease activity induced by infection is worse, and anti-infective treatment should be actively used in the early stage
.

Early control of the disease is helpful to improve the prognosis of CAPS patients
.

APS is characterized by thrombosis, manifested as deep vein thrombosis, pulmonary thromboembolism and stroke
.

The cardiac manifestations of APS are mostly valvular disease and intracardiac thrombosis, and the incidence of myocardial infarction is only 4%
.

Autoimmune diseases, including systemic lupus erythematosus, rheumatoid arthritis, systemic sclerosis and APS, are known to be chronic inflammatory states that can lead to premature atherosclerosis
.

Although the involvement of APL antibodies in the pathogenesis of APS thrombosis has been confirmed, the existence of certain cardiovascular risk factors will increase the risk of thrombosis in these patients
.

Traditional cardiovascular risk factors include obesity, diabetes, hypertension, hyperlipidemia, and smoking, which can affect the inflammatory mechanism and lipid metabolism in the body, leading to vascular damage and the development of atherosclerotic plaques
.

Recognizing and controlling these risk factors will help formulate effective treatment plans to prevent future thrombotic events
.

This case reminds us that if a patient is found to have progressive and extensive thrombosis in a short period of time, the possibility of CAPS should be considered
.

In addition, attention should be paid to controlling the traditional cardiovascular risk factors of such patients, such as diabetes, hypertension, hyperlipidemia, and smoking, which exacerbate the risk of thrombosis
.

References: [1]Parsi M, Rai M, Swaab R (March 09, 2020) A Rare Case of CatastrophicAntiphospholipid Antibody Syndrome: A Case Report and Review of TraditionalCardiovascular Risk Factors Implicated in Disease Occurrence.
Cureus 12(3):e7221.
[2]Kazzaz NM,McCune WJ,Knight JS:Treatment of catastrophic antiphospholipidsyndrome.
CurrOpin Rheumatol.
2016,28:218-227.
[3]Chinese Medical Association Rheumatology Branch.
Guidelines for the diagnosis and treatment of antiphospholipid syndrome[J] .
Chinese Journal of Rheumatology,2011,15(6):407-410.
DOI:10.
3760/cma.
j.
issn.
1007-7480.
2011.
06.
012.
[4]Huang Can, Zhao Jiuliang, Wang Qian, Li Mengtao, Tian Xinping ,.
Clinical features and prognosis of patients with catastrophic antiphospholipid syndrome[J].
Chinese Journal of Clinical Immunity and Allergy,2019,13(04):288-293.
[5],.
Catastrophic resistance Main points and progress of diagnosis and treatment of phospholipid syndrome[J].
Thrombus and Hemostasis,2020,v.
26(01):183-186.