A 66-year-old female patient with recurrent fever and with a history of Hashimoto's disease and pulmonary embolism was referred to the rheumatology department with suspected connective tissue disease. Previously, this patient had been hospitalized several times in the city hospital, where despite applied antibiotic therapy (confirmed pneumonia and urinary tract inflammation) fevers up to 38.5 C persisted. Infections such us human immunodeficiency virus, hepatitis C virus, hepatitis C virus, cytomegalovirus, toxoplasmosis, Lyme disease, and tuberculosis were excluded. Afterwards bone marrow biopsy was also taken and there was no evidence of proliferative disease.
The diagnosis of SLE was confirmed according to the European Alliance of Associations for Rheumatology (EULAR)/ACR 2018 classification criteria (fever, fluid in both pleural cavities, leukopenia, thrombocytopenia, reduced components of C3 and C4 complement, high positive dsDNA, current anti-cardiolipin antibodies in both - IgM and IgG - classes of immunoglobulins and presence of anti-beta2GPI1 antibodies).
Treatment with methylprednisolone 80 mg/day was initiated. On the second day of hospitalization due to shortness of breath, progressive decrease in blood pressure, and elevated procalcitonin serum concentration (3.61 ng/ml; >= 0.5 increased risk of sepsis), the patient was transferred to the Intensive Care Unit, where the diagnosis of severe infection with risk of septic shock was established. Empirical antibiotic treatment (blood and urine cultures were negative) and glucocorticosteroid (GC) therapy were applied (methylprednisolone 80 mg, then 60 mg) and due to an episode of atrial fibrillation, amiodarone was used to achieve a sinus rhythm.
The patient after improving the general condition was re-hospitalized in the rheumatology department where GC therapy was continued and infusions of immunoglobulins in a total dose of 135 gamma were used to further improve the general condition. In the neurological picture, flaccid four-limb weakness persisted (dominant weakness of the left upper limb and right lower limb gradually increased), and no abnormalities were found in the brain imaging by magnetic resonance imaging. An episode of unconsciousness with left eye turning and hypotension but with rapid recovery prompted the performance of a series of tests including transthoracic echocardiography and computed tomography of coronary arteries, but no significant deviations were found. Electroencephalography recording did not reveal any irritation changes typical for epilepsy. Electromyography (EMG) showed features of generalized axonal damage of peripheral nerve motor fibers, features of F wave disturbance from the right median nerve and limit values of conduction in sensory fibers of the right ulnar nerve. In addition, recording from the muscles of the upper limbs and lower limbs with features of depletion of exercise recording and an increased percentage of polyphasia. Critical illness polyneuropathy was suspected in the course of connective tissue disease and infection of unknown etiology.
In the follow-up EMG performed four weeks later, intensification of the lesions with a tendency to acute axonal polyneuropathy was observed, with a significant predominance of lesions in the nerves of the lower extremities with features of acute denervation.
The treatment with GCs was continued, methotrexate in the dose 15 mg/week was initiated, and it was planned to include hydroxychloroquine after ophthalmic contraindications was excluded. The patient was receiving low molecular weight heparin in therapeutic doses due to a history of pulmonary embolism in the course of infection, and a maintenance dose during recovery. After achieving possible improvement and a stable condition the patient was transferred to the department of rehabilitation. Then in the neurological evaluation there was described generalized weakness of trunk and limb muscle strength, which was assessed as tetraparesis with pronounced dominance of the left upper limb and global paresis of the lower limbs with the dominance of the right and abolition of tendon reflexes in the limbs. During further hospitalization, persistent lower limb allodynia with a good response to treatment with pregabalin was found.
The improvement rehabilitation program consisted of: comprehensive improvement aimed at improving self-care and developing an independent transfer. Gradual verticalization, increase of muscular strength in relief, through isometric exercises, learning to change position in the bed, and coordination exercises were used. Also a gradual improvement in muscle strength and functional status was achieved. After 6 weeks of rehabilitation the patient changed positions in bed independently and was able to cover a distance of several meters with a walker with the assistance of therapists. The next cycle of rehabilitation after 3 weeks brought a result in the form of gait improvement; initially with a high support, the patient was taught to walk up the stairs, the technique of rising after a fall, and the muscular strength and range of mobility of the left shoulder were strengthened. Significant functional improvement was noted.
At discharge, the patient walked alone for short distances (about 50 m), and for longer distances with a two-wheel support. The Barthel Index of Activities of Daily Living corresponds to the patient's current level of ability. The variables of the Barthel Index are shown in Figure 1.