Pneumococcal pneumonia is an important cause of morbidity and mortality among patients with lupus erythematosus. Therefore, a vaccination against pneumococcal infections prior to the ...immunosuppressive therapy is strongly recommended in these patients. Antimalarials are the standard first‐line systemic therapy for cutaneous lupus erythematosus (CLE). However, as many as 50% of CLE patients can be recalcitrant to this treatment and may require more intense immunosuppressive management such as for example, methotrexate, mycophenolate mofetil or azathioprine. The main aim of the current study was to assess the immunogenicity of the pneumococcal conjugate vaccine (PCV) in patients with CLE receiving hydroxychloroquine (HCQ) for at least 6 months prior to the study entry. Twenty patients with CLE but not systemic lupus erythematosus (SLE) who were receiving HCQ and five age‐ and sex‐matched healthy volunteers were included in this study. All individuals were vaccinated with 13‐valent PCV. Levels of anti‐pneumococcal capsular polysaccharide (anti‐PCP) IgM and IgG antibodies were measured before and 6 weeks after vaccination. Anti‐PCP IgM and IgG levels increased significantly in both CLE and controls upon vaccination (p < 0.0001 and p < 0.05, respectively). Ninety‐five percentage of CLE patients and 80% of healthy volunteers achieved at least 2‐fold increase in levels of anti‐PCP IgG upon vaccination. Vaccination was good tolerated in both groups. The CLE activity score before vaccination was not modified thereafter. Hydroxychloroquine does not impair immune response to PCV13. The time period when patients with CLE are receiving HCQ could be used for immunization before more intense immunosuppressive therapy would be initiated.
Introduction
The COVID-19 pandemic has changed many aspects of everyday life. Patients with primary immunodeficiency (PID) are in a particularly difficult situation. The purpose of the present study ...was to contribute to the very limited research on the everyday aspects of functioning in PID patients during the COVID-19 pandemic.
Methods
The survey included 85 adult PID patients treated with immunoglobulin replacement therapy in four reference centers for immunology. Everyday functioning of the patients as well as their opinion concerning new solutions in medical care were analyzed.
Results
During the pandemic, the percentage of patients experiencing fear/anxiety has increased from 47% to 70%. The wide dissemination of information about the SARS-CoV-2 in the media has increased anxiety in 40% of the patients. Patients diagnosed with PID were most afraid of the exposure to contact with strangers, especially in public places. As many as 67 respondents (79%) considered the introduction of restrictions concerning social functioning as good. Only every fifth person learned about the pandemic from reliable sources. Eighty three percent of the patients receiving immunoglobulin substitution experienced less fear of SARS-CoV-2 infection. The patients positively evaluated the solutions related to the direct delivery of drugs to the place of residence in order to continue home IgRT therapy. Fifty three respondents (62.5%) believed that the possibility of a remote consultation was a very good solution.
Conclusion
It is necessary to increase educational activities concerning the pandemic provided by health care professionals, as patients obtain information mainly from the media and the Internet, which adversely affects the feeling of anxiety. The pandemic, in addition to the very negative impact on patients and the deterioration of their daily functioning, has made patients appreciate their life more, devote more time to family and friends, and do things they like.
Introduction
Primary antibody deficiencies (PAD) are inborn defects of the immune system that result in increased susceptibility to infections. Despite the reduced response to vaccination, PAD ...patients still benefit from it by reducing the risk of severe infections and complications. SARS-CoV-2 vaccines are recommended in PAD patients, but their immune effects are poorly studied. Here, we analyze virus-specific T-cell responses in PAD patients after booster vaccination against SARS-CoV-2.
Patients and methods
The study included 57 adult PAD patients on long-term immunoglobulin replacement therapy (IgRT) diagnosed with X-linked agammaglobulinemia (XLA; n = 4), common variable immunodeficiency (CVID; n = 33), isotype defects or IgG subclass deficiency (n = 6), and unclassified IgG deficiency (n = 14). Of those, 49 patients (86%) received vaccination against SARS-CoV-2 using mRNA vaccine (Pfizer-BioNTech). T-cell responses were assessed at a median of 21 (13 – 30) weeks after the booster dose (mainly the third dose) using commercially available interferon-gamma release assay (IGRA) with recombinant SARS-CoV-2 spike S1 protein.
Results
Vaccinated PAD patients showed an increased (3.8-fold, p = 0.004) release of IFN-γ upon S1 stimulation. In this group, we also documented higher serum levels of anti-SARS-CoV-2 IgG (4.1-fold, p = 0.01), although they were not associated with IGRA results. Further subgroup analysis revealed very similar IGRA responses in CVID and unclassified IgG deficiencies that were 2.4-fold increased compared to XLA and 5.4-fold increased compared to patients with isotype defects or IgG subclass deficiencies (e.g., vs. CVID: p = 0.016). As expected, CVID and XLA patients showed decreased serum titers of anti-SARS-CoV-2 antibodies compared to other studied groups (e.g., CVID vs. unclassified IgG deficiency: 4.4-fold, p = 0.006). The results did not depend directly on IgRT mode or dose, number of vaccine doses and time from the last booster dose, and clinical manifestations of PAD. Interestingly, anti-SARS-CoV-2 titers were positively correlated with serum immunoglobulin levels before IgRT (e.g., for IgA: r = 0.45, p<0.001; for IgG: r = 0.34, p = 0.009) and the percentage of peripheral blood NK cells (r = 0.48, p<0.001).
Conclusions
Our results documented satisfactory
in vitro
cellular immune response in PAD patients after booster SARS-CoV-2 vaccination. Therefore, even patients with agammaglobulinemia should benefit from vaccination due to the apparent induction of cell-mediated immunity, which, together with IgRT, grants comprehensive protection against the pathogen.
Primary immunodeficiencies (PIDs) are clinically heterogeneous disorders caused by abnormalities in the immune system. However, PIDs are genetically determined and may occur at any age from early ...childhood to elderly age. Due to chronic patterns, the risk of malignancy and organ damage in patients with PIDs may affect any aspect of life, including sleep patterns. To our knowledge, the prevalence of insomnia and subjective sleep quality have not been investigated in patients with PIDs. Therefore, this pilot study was conducted to investigate sleep quality, the prevalence of sleep disturbances, and fatigue in adult patients with PIDs in Poland.
All participants were surveyed using the Athens Insomnia Scale, Pittsburgh Sleep Quality Index, Fatigue Severity Scale, and a questionnaire concerning general health and demographic data. We included 92 participants: 48 women (52.2%) and 44 men (47.8%).
Participants' mean age was 41.9 ± 13.9 years. The mean sleep duration was 7.0 ± 1.5 hours, and the mean sleep latency was 41.2 ± 53.1 minutes. Additionally, 44.6% of patients (n=41) had symptoms of insomnia and 44.6% (n=42) had poor sleep quality. Less than one-fourth (n=22; 23.9%) of the patients reported the use of sleeping pills; moreover, clinically significant fatigue was reported in 52.2% (n=48).
Our investigation provides insight into the problem of sleep disturbances in patients with PIDs. Data have demonstrated that sleeping disorders with concomitant fatigue are common in patients with PID. Further studies are needed to determine the determinants of poor sleep quality in this specific group of patients.
Data regarding the willingness of patients affected by inborn errors of immunity to accept vaccination against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection are limited. ...Therefore, this study assessed SARS-CoV-2 vaccination coverage and hesitancy in immunodeficient patients by surveying adults with primary immune deficiencies and autoinflammatory and rheumatic diseases on biologic therapy. The study was conducted from September 20, 2021, to January 22, 2022, when the primary coronavirus disease 2019 (COVID-19) vaccinations were available to all adults in Poland. We included 207 participants consecutively recruited from five referral centers (57% female; median age: 42.6 range: 18–76, standard deviation ± 14.70 years). Overall, 55% (n = 114), 17% (n = 36), and 28% (n = 57) of the patients had primary immune deficiencies, autoinflammatory diseases, and rheumatic diseases, respectively. Among the entire cohort, 168 patients (81%) were vaccinated, and 82% were willing to receive a booster dose. Patients with autoinflammatory diseases had the highest vaccination rate (94.4%). A strong conviction that it was the correct decision (72%), fear of getting COVID-19 (38%), and expert opinions (34%) influenced the decision to vaccinate. Among the unvaccinated patients, 33.3% had primary or vocational education (p <0.001). Furthermore, only 33% believed they were at risk of a severe course of COVID-19 (p = 0.014), and 10% believed in vaccine efficacy (p <0.001). They also doubted the safety of the vaccine (p <0.001) and feared a post-vaccination flare of their disease (p <0.001). Half of the unvaccinated respondents declared that they would consider changing their decision. Vaccination coverage in immunodeficient patients was higher than in the general Polish population. However, the hesitant patients doubted the vaccine’s safety, feared a post-vaccination disease flare, and had primary or vocational education. Therefore, vaccination promotion activities should stress personal safety and the low risk of disease flares due to vaccination. Furthermore, all evidence must be communicated in patient-friendly terms.
Patients with inborn errors of immunity (IEI) experience recurrent infections, autoimmunity, and malignancies. Owing to repeated medical procedures, the need for constant treatment and surveillance, ...and the unpredictable course of the disease, patients with IEI are prone to develop mental health disorders, including anxiety. In this study, we aimed to assess the prevalence and level of anxiety symptoms in adult Polish patients with IEI and explore the determinants of anxiety in this group of patients.
Data from 105 Polish patients with IEI were collected via the hospital anxiety and depression scale (HADS), brief illness perception questionnaire (B-IPQ), illness cognition questionnaire (ICQ), Pittsburgh sleep quality index (PSQI), and a questionnaire on general health and demographic data. For statistical analyses of data, the normality of distribution of quantitative data was assessed, and internal consistency of tests was investigated using Cronbach's alpha coefficient; moreover, we performed the analysis of correlations and between-group differences, and path analysis to explore causal relationships. Significance was considered at
< 0.050.
Thirty-eight (36.2%) patients had anxiety symptoms (HADS-A ≥ 8); 14 (13.3%) patients had severe anxiety (score ≥ 11), and 24 (22.9%) had moderate anxiety (score of 8-10). Patients with poor sleep quality, higher pain frequency, younger age, and no fixed income had higher anxiety scores than others. Emotional and cognitive representations of illness were positively correlated with anxiety levels. Intense anxiety was related to more negative illness perception, higher helplessness, lower illness acceptance, and lower perceived benefits.
Anxiety is common in patients with IEI. However, results indicate that it is not related to a more severe course of IEI or several comorbidities, whereas, pain frequency and poor sleep quality were identified to be important clinical factors for anxiety. Because anxiety was related to negative illness perception, psychological therapy may apply to this group of patients.
Abstract Introduction Antiphospholipid syndrome (APS) is associated with the risk of both arterial and venous thrombosis. However, it is not known which factors might determine the location of ...thrombosis. Materials and Methods To retrospectively characterize factors associated with the risk of arterial thrombosis in a cohort of APS patients. Analysis included laboratory and clinical criteria of APS, together with classical cardiovascular risk factors and the possible role of platelet integrin α2 β1 (807 C/T) and αIIb β3 (PI A1/2) genetic polymorphisms. We enrolled 163 APS patients (123 women and 40 men aged 21-75; mean age 43 years); 78 suffered from arterial thrombosis. Results There were no significant differences in the frequency or titers of different antiphospholipid antibodies with the exception of slightly increased frequency of IgG anticardiolipin antibodies (ACL) in the arterial thrombosis group. Livedo reticularis was observed significantly more often in the arterial thrombosis group, particularly in stroke patients. In univariate analysis arterial thrombosis was associated with male gender (OR-2,201; p = 0,033), arterial hypertension (OR-2,81; p = 0,002) and hypercholesterolemia (OR-3,69; p = 0,001). On multivariate analysis arterial hypertension (OR = 1,78; p = 0,008) and hypercholesterolemia (OR = 2,001; p = 0,002) remained as independent risk factors for arterial thrombosis. Platelet glycoprotein polymorphisms studied did not show any significant associations with arterial thrombosis in APS patients. Conclusions Among APS patients those with ACL IgG antibodies, having livedo reticularis, and suffering from hypertension an hypercholesterolemia are at the increased risk of arterial thrombosis.
Common variable immunodeficiency (CVID) is the most clinically significant primary antibody immunodeficiency recognized in adulthood. Previously published data have shown an average diagnostic delay ...of 10 years for Polish adult patients with CVID. In the current study, we aimed to analyze the current diagnostic delay of adult patients with CVID in Poland. To this end, we identified patients from four immunological centers specialized in the care of adult patients with primary immunodeficiencies (PID). Demographic and clinical data of patients were collected using an internet database. We identified 103 adult patients (F:M 44.7%:55.3%) in Poland with CVID. The median age at onset of symptoms was 24 (0-66), 33 (4-70) at diagnosis, and 37 (18-73) years at the time of analysis. The median diagnostic delay for the entire study population was 6 (0-57) years. However, this delay was higher in patients with symptom onset before the year 2000 than after the year 1999 15 (0-57) vs. 3 (0-19) years;
< 0.001. Comparing patients (median ≤ 6 years,
= 53) with short diagnostic delay (SDD) and those (median > 6 years,
= 50) with long diagnostic delay (LDD), the LDD group had a statistically significant higher incidence of infections of the lower respiratory tract before diagnosis (90.0 vs. 71.70%). During the entire observation period, cytopenias (44.00 vs. 22.64%), granulomatous lesions (28.00 vs. 11.32%), and solid tumors (14.00 vs. 1.89%) were significantly more frequent in the LDD group. In conclusion, we found a significant reduction in the median diagnostic delay in Polish CVID patients with disease onset in the last two decades.
The majority of primary immunodeficiencies (PIDs) are antibody deficiencies (PADs), and not all of them are rare diseases; As an example, Caucasian individuals suffer from selective IgA deficiency at ...a frequency of 1:500.In addition to infections, symptomatic patients with PAD are more likely to develop neoplastic, autoimmune, and allergic diseases. In the event that PAD is neglected or delayed for more than ten years, complications develop, eventually resulting in death. No studies have been conducted to devise and report detailed ready-to-use protocols for managing PAD to date.This study aimed to propose protocols and guidelines for the adult PAD patients’ standard care. Preparing the protocol, we considered the frequency and type of laboratory tests, imaging, endoscopic examinations, specialist consultations, and standardized recommendations for further care in the place of residence. As a result of the proposed monitoring scheme, patients can be provided with complete care in terms of their underlying conditions and comorbidities, as well as early detection of complications. This protocol will serve as a guide for physicians dealing with these patients and enable comparisons of patient groups across a variety of treatment centers, even far away from each other. A national consultant in the field of clinical immunology verified the protocol mainly developed by Polish experts from reference immunology centres for adults.
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