*For medical professionals only
Do not ignore any details of the patient's onset
Synopsis
A 31-year-old female patient [1] who was initially taken to the emergency room for pneumonia and was admitted to the hospital to have hypertension with new onset of enlarged heart. The doctor then perfected the admission examination to find out the cause of high blood pressure and heart disease.
However, during this time, the patient developed acute kidney injury (AKI) with acute and chronic obstruction of the abdominal aorta. Such a complex and dangerous condition, can you guess what the disease is?
Case profile
The patient was presented to a hospital in Australia for 6 weeks due to respiratory symptoms. He is in good health, with a history of one previous medical miscarriage and two pregnancies.
Physical examination at admission: blood pressure 198/120 mmHg, heart rate 100 beats/min. There are bronchial breath sounds on the right side of auscultation of the lungs, and no abnormalities are found on the rest of the examination. Chest X-ray at admission shows new-onset cardiac enlargement, diffuse lesions in both mid- and lower-middle areas, with multiple lateral interstitial lines and pulmonary vein congestion (Figure 1). CT chest shows ground glass-like shadow with mediastinal lymph node lesions.
Fig. 1 Chest x-ray at admission
Laboratory tests:
White blood cell count (WBC) 21.3×109/L, C-reactive protein (CRP) 212 mg/L, and N-terminal b-type natriuretic peptide precursor (NT-proBNP) 16788ng/L were all elevated, and creatinine was 72 mol/L.
Find out, what is the soaring indicator?
Seeing this, do you still think that this is an ordinary pneumonia patient, and the patient has indeed started to take azithromycin and ceftriaxone to treat pneumonia. But what's confusing is what this soaring blood pressure and NT-proBNP value is all about?
The patient then improved echocardiography (TTE) and renal vascular ultrasonography, showing mild mitral regurgitation and severe systolic dysfunction with an ejection fraction of 32%. Doctors treat patients with diuretics such as ramipril, spironolactone, and bisoprolol.
However, there are also some tests that have caught the attention of doctors! Initial renal vascular ultrasound did not show any renal artery stenosis. However, aortic occlusion is accidentally found below the superior mesenteric artery.
Abdominal CT scan on day 4 of admission confirmed that there was a filling defect of 7 cm in the abdominal aorta below the left renal artery, and the right renal artery was significantly occluded. The distal abdominal aorta, thoracic vessels, pelvic vessels, and femoral vessels have extensive collateral circulation (Figure 2), suggesting chronic pathology. And the patient has previous symptoms of lower extremity claudication.
In addition, the patient's renin level was elevated to 116 mU/L, consistent with renovascular hypertension.
Fig. 2 Abdominal CT angiography
At the same time, the patient developed bilateral earache associated with pinna discoloration (Figure 3), which she had also experienced before.
Fig. 3 Recurrent polychondritis
The disease has progressed here, and the initial doubts have not been solved, and new symptoms have appeared one after another. But in fact, it also provides more basis for doctors to diagnose diseases.
Doctors initially diagnosed large-vessel vasculitis and recurrent polychondritis, and the patient began treatment with azathioprine and methylprednisolone pulses, which effectively improved the patient's inflammation of the pinna. Therapeutic anticoagulation is performed with low molecular weight heparin and screening for causes of vasculitis and thrombosis is performed.
The improvement of the patient's ear symptoms seems to mean that the condition has begun to develop for the better, and it has not been thought that the joy of seeing the effect at first sight has not yet had time to feel, and the patient's kidneys have begun to pick the picks.
Confusing, said to change the disease
On the 5th day of admission, the patient developed AKI, and creatinine increased from 72 mol/L at the time of admission to 124 mol/L. And without stopping, renal function continued to deteriorate, creatinine increased to 328 mol/L, and progressed to anuria and urinary retention on day 10. Renal vascular ultrasonography was performed again, and the results showed that aortic obstruction may extend to the abdominal axis, and there is no blood flow in the renal vessels, resulting in worsening renal function.
What is the situation with this sudden AKI? Everyone was dumbfounded. Did previously diagnosed vasculitis cause AKI? After all, this is the only diagnosis that can be reached at present. Could drugs also be the cause of AKI? Of course, there is another object of suspicion: contrast agents.
Only one by one can be excluded. After doctors discontinued nephrotoxic drugs such as diuretics and angiotensin-converting enzyme (ACE) inhibitors, the patient's kidney function still showed no signs of recovery, and the "suspicion" of the drug was ruled out.
In addition, although the use of contrast agents may exacerbate kidney damage, especially in the case of decreased renal perfusion and the use of nephrotoxic drugs. But AKI occurs within 24 hours of using the contrast agent, so it is unlikely that the contrast agent is the main cause of the initial decline in kidney function.
Then only the "major suspect" of vasculitis remains.
Pulling away the clouds, the real culprit behind the curtain was TA
The patient's anticoagulation was changed to heparin infusion and he was urgently transferred to a tertiary hospital for re-examination in interventional radiology, vascular surgery, nephrology, cardiology, and rheumatology and immunology. Due to the excessive risk, there was no intervention for thrombosis conditions such as stenting, thrombolysis, or surgical treatment. During the tertiary hospital, the patient underwent a fluorodeoxyglucose (FDG)-positron emission tomography (PET) scan, and to everyone's surprise, the examination showed no evidence of large-vessel vasculitis.
What the hell is going on, all the previous evidence points to vasculitis of macrovascularity, and the precision machine coldly rejects this diagnosis. Without a definitive diagnosis, it is even more impossible to explain the patient's AKI, which makes everyone take a breath of cool air. How can this be done?
Miscarriage, AKI, soaring blood pressure and NT-proBNP... The truth seems to be close at hand.
Soon after, the patients were positive for lupus anticoagulants (LA), anticardiolipin antibodies (ACA), and anti-beta2 glycoprotein 1 antibodies (anti-beta2GP1 antibodies). These all point to a disease called catastrophic antiphospholipid syndrome (CAPS).
Disease knowledge window
1. What kind of disease is CAPS?
To understand CAPS, you first need to understand what antiphospholipid syndrome (APS) is. APS, also known as Hughes syndrome, is an autoimmune disorder characterized by a venous or arterial thrombosis that is positive for antiphospholipid antibodies (aPL), or has an obstetric disorder (such as miscarriage, fetal death, and preterm birth).
CAPS is an autoimmune disorder characterized by intravascular thrombosis and/or pathological pregnancy, accompanied by positive aPL. It is a special type of APS characterized by rapidly progressive multi-organ thrombosis and concomitant high-titer aPL, which can lead to catastrophic clinical outcomes [3].
Early diagnosis and therapeutic intervention by clinicians is important in rescuing CAPS from short-term multi-organ failure, and suggests the potential value of anti-inflammatory measures such as hormones and immunotherapy.
The clinical manifestations of CAPS are diverse because they originate from organ systems affected by thrombosis. The kidneys are the most common organs affected, with predominantly renal insufficiency and proteinuria. Cardiac problems are also common, manifesting as left ventricular dysfunction, valvular insufficiency, and less commonly myocardial infarction.
2. What are the key points of DIAGNOSIS of CAPS?
History of APS or presence of aPL, such as LA, ACA, and persistent positive anti-beta2GP1 antibodies.
Duration of involvement of various organs
There is histopathological evidence of small thrombosis.
Other manifestations of multi-organ thrombosis and /or microthrombosis.
3. How is CAPS treated?
Anticoagulation, glucocorticoids, intravenous immunoglobulins, and/or plasmapheresis triple therapy are recommended treatments.
Summary of cases
The case is of a young, healthy woman who was diagnosed with CAPS at the time of the first onset of illness. About half of all APS initially came in the form of CAPS. The patient was screened again for aPL 16 weeks after onset and the patient remained positive for anti-beta2GP1 antibodies. Thus, the diagnosis of previous CAPS was confirmed. The patient later underwent hemodialysis and continued treatment with therapeutic anticoagulation and glucocorticoids.
bibliography:
[1] Kwak S, Green M. Rare yet catastrophic presentation of undiagnosed antiphospholipid syndrome[J]. BMJ Case Rep 2022；15
Wang Wenqiang, Li Zhongxin, Kong Xiaolu, et al. Diagnosis and research progress of antiphospholipid syndrome[J]. Massage and Rehabilitation Medicine, 2021, 12(10):3.
Yang Xin, Li Dengju. Key points and progress in diagnosis and treatment of catastrophic antiphospholipid syndrome[J]. Thrombosis and Hemostasis, 2020, 26(1):4.
Source: Rheumatic Immunity Channel of the Medical Community
Author: Fly First
Review: Chen Xinpeng Deputy Chief Physician
Editor-in-charge: Wang Hang
Proofreader: Zang Hengjia
Plate making: Xue Jiao