The deficiency of alpha-1 protease inhibitor, or alpha-1-antitrypsin (A1AT), predisposes to chronic lung diseases and extrapulmonary pathology. Besides classical manifestations, such as pulmonary emphysema and liver disease, alpha-1-antitrypsin deficiency (A1ATD) is also known to be associated with granulomatosis with polyangiitis (GPA or Wegener's granulomatosis). The aim of our study was to evaluate the frequency of allelic isoforms of A1AT and their clinical significance among GPA patients. Detailed clinical information, including Birmingham Vasculitis Activity Score (BVAS), incidence of lung involvement, anti-proteinase 3 (PR3) antibodies concentrations, and other laboratory data were collected in 38 GPA patients. We also studied serum samples obtained from 46 healthy donors. In all collected samples A1AT phenotyping by isoelectrofocusing (IEF) and turbidimetric A1AT measurement were performed. Abnormal A1AT variants were found in 18.4% (7/38) of cases: 1 ZZ, 4 MZ, 2 MF, and only 1 MZ in control group (2%). The mean A1AT concentration in samples with atypical A1AT phenotypes was significantly lower (P = 0.0038) than in normal A1AT phenotype. We found that patients with abnormal A1AT phenotypes had significantly higher vasculitis activity (BVAS) as well as anti-PR3 antibodies concentration. We conclude that A1AT deficiency should be considered in all patients with GPA.
BACKGROUND: The COVID-19 pandemic poses a particular threat to patients suffering from immunoinflammatory rheumatic diseases. New coronavirus infection has been found to be accompanied by the development of a wide range of extrapulmonary clinical and laboratory manifestations, which are characteristic of a number of immunoinflammatory rheumatic diseases.
AIM: To evaluate the features of the clinical course of immunoinflammatory rheumatic diseases in patients who underwent new coronavirus infection.
MATERIALS AND METHODS: The clinical course of immunoinflammatory rheumatic diseases was analyzed in 324 patients who underwent new coronavirus infection from March 2020 to February 2021 and were treated at the Clinical Rheumatology Hospital No. 25, Saint Petersburg, for exacerbation of the underlying disease.
RESULTS: Analysis showed that the risk factors for severe new coronavirus infection in patients with immunoinflammatory rheumatic diseases were: age over 60, comorbidities, use of prednisolone in a dose greater than 12,5 mg, and ESR values 40 mm/hour before the development of new coronavirus infection. There was no effect of immunosuppressive and biological therapy on the severity of the course of viral infection. There was no effect of immunosuppressive therapy and biological therapy on the severity of the course of viral infection in patients with immunoinflammatory rheumatic diseases. The development of the postinfectious syndrome was observed in 1/4 of patients, which was characterized by the formation of postinfectious arthritis in 3,6% of patients, transformation of undifferentiated arthritis into various rheumatic diseases in 49% of patients (more often into early rheumatoid arthritis), as well as exacerbation of the underlying disease in 83,4% of patients with an advanced stage of rheumatoid arthritis. In patients with mixed connective tissue disease, there was a significant increase in immunologic activity due to antinuclear factor (up to a maximum of 1:163 840). Clinical cases of the development of arthritis associated with viral infection and the debut of rheumatoid arthritis after an new coronavirus infection are presented.
CONCLUSIONS: New coronavirus infection in the cohort of patients with immunoinflammatory rheumatic diseases observed in the Clinical Rheumatology Hospital No. 25, Saint Petersburg, proceeded in the variant of medium severity in half of patients, initiated the development of lung lesions in 68,6% of patients, arthritis associated with viral infection in 3,6% of patients, immunoinflammatory rheumatic diseases which transformed from undifferentiated arthritis in 49% of cases and exacerbation of the main disease in an overwhelming number of patients. Patients with immunoinflammatory rheumatic diseases have a high risk of adverse outcome of new coronavirus infection, especially in cases of unstable course of the disease or exacerbation of this group of diseases.
The review presents the results of scientific studies devoted to the features of the course and outcomes of a new coronavirus infection COVID-19 in patients with immuno-inflammatory rheumatic diseases (IIRD). It was noted that the risk of developing COVID-19 for patients with IVR, apparently, is similar to the population or slightly increased and mostly depends on the presence of established risk factors for its severe course (old age, obesity, diabetes mellitus, cardiovascular diseases). Patients receiving long-term immunosuppressive therapy and high doses of glucocorticoids may have a long period of positive viral replication and isolation of a viable virus, which requires dynamic monitoring of such patients and correction of anti-rheumatic therapy. The issues of post-COVID-19 joint syndrome, which can occur within the framework of post-viral arthritis or be the debut of an immuno-inflammatory rheumatic disease, are highlighted. The draft recommendations of the All-Russian Public Organization Association of Rheumatologists of Russia on the management and temporary recommendations of V.A. Nasonova Research Institute of Rheumatology for vaccination of patients with rheumatic diseases in the conditions of the COVID-19 pandemic are presented.
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