Our patient is a 57-year-old woman. She had been completely healthy until 1 year before hospitalization, when she was diagnosed with asthma and chronic sinusitis. Progressive discomfort and tingling in the soles and later in the palms appeared 3 months before admission. Later, pain and mild weakness in the feet and hands emerged, and a rash on the legs occurred. Two weeks before hospitalization, she noticed worsening of asthma and sinusitis, worsening of painful sensations on the soles and palms, malaise, languor, pain in the muscles and joints, and gait disturbances due to previous symptoms.
On admission, she complained of malaise and fatigue, myalgia, and arthralgia. She had a body temperature of 37.5 °C, serous nasal discharge, and a purpuric rash on her legs. Muscle strength assessed using the Medical Research Council (MRC) rating scale was reduced globally (proximal upper limbs, MRC grade 4/5; proximal lower limbs, 3/5; distal upper limbs, 4/5; distal lower limbs, 3/5). An examination of her sensory systems showed paresthesia, dysesthesia, and allodynia in her soles and palms. In the initial laboratory, the absolute number of eosinophils was 9.95 × 109/L, and they made up 50% of white blood cells. Creatine kinase, lactate dehydrogenase, aspartate aminotransferase, and alanine aminotransferase (2901 IU/L, 1137 IU/L, 97 IU/L, and 137 IU/L, respectively), as well as erythrocyte sedimentation rate and C-reactive protein, were elevated.
On the third hospital day, she presented with left-sided hemiparesis. The first brain computer tomography (CT) had been normal, but control brain CT performed 48 h after symptom onset showed a subacute ischemic lesion in the right basal ganglia region (Fig. 1a). Head magnetic resonance imaging (MRI) confirmed this finding (Fig. 1b, c). Doppler ultrasonography of the neck arteries and transcranial Doppler were normal. Cardiological evaluation, troponin level, electrocardiogram, 24 h electrocardiogram, and cardiac ultrasound were also normal.
Coagulation status was in the reference range. Studies of inherited (factor V Leiden, prothrombin G20210A, protein C and S deficiency, antithrombin deficiency, methylenetetrahydrofolate reductase/MTHFR C677T) and acquired (lupus anticoagulant, IgM and IgG anticardiolipin, and anti-β2 glycoprotein 1 antibodies) thrombophilia were negative. Electromyoneurography showed the presence of myopathy and sensorimotor, axonal polyneuropathy in the upper and lower extremities. Metabolic and paraneoplastic causes of polyneuropathy were excluded. Hematological and infectious (Trichinella spiralis, Toxoplasma gondi, Toxocara canis, Brucella spp., Echinococcus, Cysticercus, and other helminthic infections, HIV, etc.) causes of eosinophilia also were excluded. The immunological analysis revealed elevated pANCA antibodies. Estimated urinary protein excretion was 2.3 g/day at admission (serum creatinine level 47 μmol/L; urine sediment was normal). CT of the thorax showed a normal finding.
Based on the presence of asthma, sinusitis, polyneuropathy, hypereosinophilia, and positive pANCA antibodies, EGPA was diagnosed. Intravenous corticosteroid therapy with methylprednisolone (starting 1000 mg/day for 5 days followed by reduced doses) was administered for 2 weeks. After the third dose of intravenous methylprednisolone, the absolute number of eosinophils was 0.55 × 109/L (7.8% of white blood cells), and the estimated urinary protein excretion was 1.3 g/day. Treatment was continued with oral prednisone therapy (1 mg/kg/day) in combination with oral azathioprine (2 mg/kg/day). During hospitalization, improvement of physical and neurological status and further normalization of blood and urine findings were noted. After 45 days of hospitalization, she was discharged with mild left-sided hemiparesis but was able to walk without assistance. Her therapy on discharge was prednisone (1 mg/kg/day in tapering doses), azathioprine (2 mg/kg/day), and acetylsalicylic acid (100 mg/day).
Forty days later, during the outpatient control, global weakness of the right extremities and dyspnea were noticed. Due to clinical deterioration, she was hospitalized again. CT of the brain showed a subacute ischemic lesion in the left region of the basal ganglia (Fig. 1d) and a chronic ischemic lesion in the region of the basal ganglia on the right side (documented earlier, Fig. 1a). Control eosinophils, CRP, and 24 h proteinuria were in the normal range. Elevated d-dimer value of 9.87 mg/L (cutoff value < 0.5 mg/L) was observed, while the rest of the coagulation status was in the normal range. Control analyses of lupus anticoagulant, IgM and IgG anticardiolipin, and anti-β2 glycoprotein 1 antibodies were negative, while the follow-up level of pANCA was decreased. Thorax CT revealed segmental pulmonary embolism in the right lower lobe. Cardioembolism, atrial or interventricular septal defect, and deep vein thrombosis were ruled out by ultrasound of the heart and peripheral veins. Subcutaneous low-molecular-weight heparin (enoxaparin sodium at dose 6000 units every 12 h) was administered. Assuming that complete remission of the disease had not been achieved previously, intravenous methylprednisolone therapy (1000 mg/day) was administered for 5 days, followed by oral prednisone (1 mg/kg/day) which was gradually reduced, oral coumarin anticoagulant therapy was introduced, and pregabalin was given for neuropathic pain. The patient has entered stable remission. During the 3-year follow-up, control neurological findings have shown the maintenance of mild left side hemiparesis accompanied by action left foot dystonia, oral prednisone has been reduced to a maintenance dose of 10 mg/day, and azathioprine therapy has been slowly discontinued, while neuropathic pain has been well controlled.
