Search

Background/Objectives: Inborn errors of immunity (IEI) encompass various congenital disorders, resulting in immunity defects and recurrent infections. Home-based subcutaneous immunoglobulin replacement therapy (scIgRT) is the best treatment option for those with primary antibody deficiency (PAD). However, the lack of standardized procedures in patient training remains a challenge. Our study investigates nurses' practice and perspectives, aiming to identify areas for improvement in at-home scIgRT practice. Methods: We prepared a structured survey regarding scIgRT, including needle choice experience and perception of adverse events, and distributed it among qualified nurses involved in patient training and scIgRT supervising. Results: We included 56 nurses with a median age of 50 years. Among them, 67.9% represented adult care providers, while 32.1% supervised IgRT in children. Most respondents (83.9%) used the classic or assisted with hyaluronidase scIgRT preparations. Single-channel needles were administered most commonly (85.7%). The needle length was mostly chosen solely by a nurse (57.1%) or in cooperation with the patient (23.2%). Next, 9 mm and 12 mm needles were used most often (92.9% and 78.6%, respectively). As expected, the 6 mm needle was more frequently applied for children compared to adults (n = 16, 88.9% vs. n = 11, 28.9%, p < 0.001), while 12 mm was primarily used in adults (n = 35, 92.1% vs. n = 9, 50.0%, p < 0.001). Visual skin fold assessment was the basis for the needle selection (58.9%), followed by the injection site rule (26.8%) or a choice between two available needle types for thinner or thicker patients (25.0%). Results of this survey indicate that, according to nurses' opinions presented in this survey, the needle length could be associated with local scIgRT adverse events, such as side needle leakage or local burning. Yet, it was likely unrelated to general adverse signs, such as headaches or dizziness. Most respondents (66.1%) indicated that, even if local adverse events occur, patients are reluctant to change scIgRT preparation or needle length. Most participants (69.6%) reported that the optimal administration technique needs to be discussed with the patient before and during scIgRT. Conclusions: This study sheds light on scIgRT practice in Poland, emphasizing deficiency in needle selection technique. Future research should focus on standardized training and advanced needle selection procedures on patient outcomes, investigating the correlation between needle strategies and adverse events, as well as the effectiveness of scIgRT. [ABSTRACT FROM AUTHOR]

Background. Certain mediators, such as soluble growth factors and cytokines, among others, are implicated in the immunopathogenesis of systemic sclerosis (SSc). Objectives. This study aimed to examine the association between serum levels of vascular endothelial growth factor (VEGF), interleukin-8 (IL-8), interferon alpha (IFN-a), and basic fibroblast growth factor (bFGF) and the clinical presentation and course of SSc. Materials and methods. This longitudinal, observational study included 43 patients with SSc and 24 healthy subjects. Serum concentrations of VEGF, IL-8, IFN-a, and bFGF were measured at baseline in patients previously treated for SSc. Medical history of patients was analyzed retrospectively at the time of cytokine measurement to infer clinical correlations, and during follow-up for a median of 5 years, assessing the incidence of death or cancer. Results. The bFGF and IFN-a concentrations differed between SSc patients and controls (p < 0.01). In turn, organ involvement and SSc phenotypes did not impact studied cytokine concentrations, similar to systemic steroid and/or immunosuppressant use at enrollment. However, we have documented a positive correlation between the current oral steroid dose and serum levels of IL-8 and bFGF. Furthermore, patients with a VEGF level =95.7 pg/mL and IFN-a level =3.6 pg/mL required cyclophosphamide therapy more often, currently or in the past (approx. 3-fold and 4-fold, respectively). Substantially elevated VEGF and IFN-a concentrations at baseline were associated with higher cancer occurrence (n = 4) during follow-up, while elevated circulating IL-8 level was associated with an increased risk of death (n = 9). Conclusions. The SSc group was characterized by higher serum concentrations of bFGF and IFN-a compared to healthy controls. Patients treated with cyclophosphamide or receiving higher systemic steroid doses, thus suffering from a more severe disease type, had increased cytokine levels. Elevated circulating IFN-a and VEGF levels might be correlated with cancer, whereas raised IL-8 levels may be associated with an increased risk of death. However, further research is needed to verify our findings. [ABSTRACT FROM AUTHOR]

Background: 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. Methods: Data from 105 Polish patients with IEI were collected via the hospital anxiety and depression scale (HADS), brief illness perception questionnaire (BIPQ), 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 betweengroup differences, and path analysis to explore causal relationships. Significance was considered at p < 0.050. Results: 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. Discussion: 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 FROM AUTHOR]

Erythema multiforme is extremely rare after topical drugs. We described a case of 57-year old female patient with an eight years history of systemic lupus erythematosus (SLE), presented with erythema multiforme after topical use of a skeletal muscle relaxant (idrocilamide). Skin changes progressed to generalized erythroderma dispite high dose systemic steroid treatment but rapidly improved after plasmapheresis. [ABSTRACT FROM AUTHOR]

Competing Interests: The authors declare no conflict of interest.

A 27-year-old male with a six-year history of hypereosinophilic syndrome (HES) presented with a native mitral valve thrombus, despite therapeutic oral anticoagulation. The thrombus was removed, the mitral valve replaced, and subsequent oral anticoagulation maintained at a higher level (INR 3.5). The patient developed two recurrences of mitral valve thrombosis requiring urgent reoperations, and died shortly after the second intervention. A scanning electron microscopy analysis of the native mitral valve thrombus removed during the first cardiac surgery revealed tightly packed thin fibrin strands forming fuzzy irregular structures, with areas of an almost solid fibrin clot. The fibrin networks indicated a heightened thrombin generation, and may account for a diminished susceptibility to intrinsic fibrinolysis. In conclusion, the unfavorably altered compact structure of the fibrin-rich thrombus, which formed despite adequate anticoagulation, might in part explain the recurrent valvular thrombosis. It may also represent a novel prothrombotic mechanism that operates in HES.

Patients with a suspicion of non-Hodgkin lymphoma (NHL) represent a diagnostic challenge for clinicians, because they may present with a variety of symptoms. We present a series of 5 patients in whom persistent fever was one of the earliest symptoms of NHL.