Sinusitis patients with cough, myalgia, and finally cerebral infarction are admitted to the hospital, but it is not so simple!

*For medical professionals only to read for reference Raising awareness of non-specific symptoms can light up the patient's life! Abstract: A 69-year-old male patient had a history of bladder cancer, asthma, and stage IIIa chronic kidney disease
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Initial laboratory tests showed increased white blood cells (WBC), and the clinical manifestations were discomfort such as cough, rhinorrhea, and sinus congestion, along with myalgias and arthralgias
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So the doctor before admission suspected that this series of symptoms was caused by the patient's infection with the virus, and gave symptomatic and supportive treatment
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But the patient's condition not only did not improve, but two days later, he also suffered from jaw and cheek pain, and finally even had a supraventricular tachycardia (SVT) attack.
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The whole course of the disease can be described as complicated and dangerous~ I don't know you on the other side of the screen, at this moment Is there a vague answer in your heart? Next, let the editor take you closer to this case and share the secret~ Simple virus infection? The patient was not hospitalized when the above symptoms appeared, but only went to see a doctor and completed laboratory tests
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Out-of-hospital laboratory examination showed [1]: C-reactive protein 6.
04mg/dl ↑; rheumatoid factor 25IU/ml ↑; creatine kinase 1465U/l ↑; urine protein (++); hematuria (+++); eosinophilia Granulocytes were 11.
17×109/L↑, accounting for 45% of the total number of white blood cells
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Considering that the patient has a history of kidney disease, the doctor did not think much after seeing the test results, but simply treated the patient according to the viral infection
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The treatment did not work, however, and the patient had increased jaw and cheek pain when chewing two days later, and difficulty chewing six days later
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Shows that a simple "viral infection" does not explain the above symptoms
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Then what would it be? Difficulty chewing, eosinophilia.
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Could it be giant cell arteritis? The diagnosis suddenly appeared in front of the doctor's eyes, so he immediately performed arteritis examination and kidney biopsy on the patient, trying to confirm his diagnosis
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But one wave has not settled, and another wave has arisen.
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Layer by layer, who is the real culprit? After a kidney biopsy, the patient complained of weakness and numbness in his right arm and was rushed to the emergency room
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Sure enough, the condition worsened again.
In the emergency room, the patient had an SVT attack with a heart rate of 190s/min, which lasted for 5 minutes
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 Soon, the patient was further refined in the emergency department, with CT angiography of the neck showing no vascular stenosis, and MRI showing a lacunar infarction (Figure 1)
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Figure 1 MRI tracking image shows bright periventricular hyperintensity consistent with a lacunar infarction (red circle, top row)
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The same hypodense lesions on the corresponding ADC (apparent diffusion coefficient) images confirmed limited diffusion (red circles, bottom row)
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 Good guy, first there was a problem with the respiratory system, and then the heart was also affected, and now the examination shows the patient's cerebral infarction, which is really a "serial surprise"! After emergency treatment, the patient was transferred to the hospital ward.
At this time, the patient's vital signs were stable, and the physical examination showed no obvious abnormality
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Laboratory examinations on admission showed that eosinophils were lower than before, but the value was still higher than normal, at 6.
34×109/L, accounting for 32% of the total number of white blood cells; chest X-ray, echocardiography and magnetic resonance angiography were all No abnormality; myeloperoxidase (MPO), anti-neutrophil cytoplasmic antibody (ANCA), and protease 3 (PR-3) were all negative
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Table 1 Laboratory values ​​of the patient out-of-hospital and during admission At this time, the results of a renal biopsy previously performed out-of-hospital were also available, showing pauci-immune complex glomerulonephritis with massive eosinophilic infiltrates and small interstitial fibrosis or renal tubular atrophy
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Figure 2 Glomerulus showing extracapillary proliferation of epithelial cells, consistent with cellular crescents (A, black arrows)
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Focal fibrinoid necrosis (A, red arrow) is also present
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Overall, 45% of glomeruli showed necrosis and/or cellular crescent damage
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The tubulointerstitium contains marked eosinophilic infiltration (B, H and E staining)
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 All the above symptoms and examination results point to the same disease - eosinophilic granulomatosis with polyangiitis (EGPA)
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What is EGPA? EGPA is a rare, autoimmune disease that can involve multiple systems in the body.
It is mainly manifested as eosinophilia, infiltration, and necrotizing granulomatous inflammation of small and medium blood vessels in peripheral blood and tissues.
It belongs to ANCA-related diseases.
Vasculitis [2]
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 EGPA can involve multiple organs such as sinuses, lungs, skin, nervous system, heart, gastrointestinal tract, kidneys, etc.
The vast majority of patients have asthma and/or allergic rhinitis
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It is currently believed that the pathogenesis of EGPA is ANCA-mediated vascular wall damage and eosinophil infiltration
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ANCA-mediated EGPA is mainly involved in the kidneys, but also has purpura, alveolar hemorrhage, sinusitis, etc.
, and the incidence of peripheral neuropathy is higher; while EGPA mediated by eosinophil infiltration is mainly involved in the lungs, and the heart Involvement (eg, pericarditis and cardiomyopathy), pleural effusion, and fever were more frequent
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 The current diagnostic criteria of EGPA mainly refer to the classification criteria proposed by the American College of Rheumatology (ACR) in 1990, including clinical manifestations, laboratory examinations, imaging examinations, and pathological biopsy
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The 6 classification criteria include: (1) asthma-like symptoms (or wheezing attacks); (2) eosinophilia (≥10% or absolute value ≥1.
5×109/L); (3) single or multiple nerves Lesions; (4) non-fixed pulmonary infiltrates; (5) sinusitis; (6) extravascular eosinophilic infiltration
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 Meet 4 or more can diagnose EGPA
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The patient in this case presented with four of six criteria: asthma, eosinophils >10% of total leukocytes, neuropathy (cerebral infarction, a rare CNS disorder associated with EGPA), and extravascular eosinophilic infiltration
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Table 2.
The EGPA classification criteria developed by the ACR in 1990.
The timely treatment after the cloud was cleared.
After the diagnosis was clarified, the patient began to receive intravenous methylprednisolone (1g per day) for 3 days, and then after high-dose steroid pulses, he Gradually switch to 1 mg/kg/d of prednisolone
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Given the patient's history of bladder cancer, the patient received rituximab 1000 mg infusion instead of standard cyclophosphamide to avoid bladder toxicity from cyclophosphamide
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Rituximab was given again two weeks later, and the patient's condition improved
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 After high-dose steroid pulses, the patient had no recurrence of SVT
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After 6 months of rituximab treatment, there was no episode of SVT, and the eosinophil count was less than 1.
5% of the WBC count, and the glomerular filtration rate (GFR) changed from that before treatment.
49.
7ml/min increased to 69.
1ml/min
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Thanks to the doctor's timely and clear diagnosis, the patient's condition was finally brought under control without causing further damage
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 Summary EGPA is different from other vasculitis in that it is the earliest and most likely to involve the respiratory tract and lungs.
Most of the first symptoms are wheezing, myalgia and sinusitis
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The patient in this case reported his jaw pain symptoms to the doctor in time, which prompted the improvement of the follow-up examination, so he received timely treatment
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But in fact, due to the non-specificity of these symptoms, it is easy to be misdiagnosed in clinical practice.
Many patients are often accompanied by multi-system involvement of the whole body when they are diagnosed, causing irreversible organ damage, which brings difficulties to treatment and affects The prognosis of the patient's disease
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Therefore, improving the awareness of EGPA, especially how to detect EGPA patients early in the refractory asthma population, is the key to early diagnosis, standardized treatment, improvement of curative effect, and reduction of morbidity and mortality of EGPA
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Reference: [1] Huckenpahler AL, Iqbal H, Gallan A, Jha P.
Eosinophilic granulomatosis with polyangiitis: an unusual cause of stroke.
BMJ Case Rep.
2022 Apr 5;15(4):e236844.
[2]Eosinophilic granulomatosis Multidisciplinary expert consensus writing group for the diagnosis and treatment of eosinophilic granulomatosis with polyangiitis [J].
Chinese Journal of Tuberculosis and Respiratory Medicine, 2018, 41(7):8.