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Introduction
Granulomatosis with polyangiitis (GPA) is a necrotizing granulomatous inflammation usually involving the upper and lower respiratory tracts and a necrotizing vasculitis mainly involving small and medium-sized vessels [1]. The disease can produce a varied clinical picture depending on the system most involved and the size of the necrotic lesions within it [2]. The disease often begins with general symptoms, as well as symptoms from the upper respiratory tract, lungs and kidneys (in <20%). Its course can vary from a slow, mild course to rapid progression with life-threatening changes in multiple organs [1]. Symptoms involving the respiratory system may also vary in severity. They include cough, also with expectoration of purulent secretions, hemoptysis, dyspnea or chest pain [3]. The disease may involve mucosal ulceration, paranasal sinusitis and otitis media not amenable to conventional treatment. Granulomatous lesions can cause bronchial deformation and stenosis [3]. The disease is often accompanied by necrotizing glomerulonephritis. In addition, the disease can affect the eye, nervous system, skin, gastrointestinal tract, or musculoskeletal system [3, 4].
The diagnosis of the disease is based on a typical clinical picture, histopathological examination and the presence of antineutrophil cytoplasmic antibodies (ANCA) [5]. The diagnosis of the disease is possible thanks to the ACR/EULAR classification criteria (2022) for granulomatosis with polyangiitis (GPA) [6]. The treatment of the disease depends on the clinical picture and its severity. In an acute phase of the disease, remission-inducing treatment is used and once remission is achieved, maintenance treatment is applied [1]. Due to the application of immunosuppressive treatment, since its discovery the disease has changed from fatal to chronic and relapsing condition [3].
Relapses and exacerbations of the disease can affect patients’ functioning in their daily life. The unpredictability of the course of the disease can also aggravate the stress experienced by patients [7]. In addition to somatic complaints, a diagnosis of rheumatic disease may involve the need to change life plans, including even loss of work and position in society or financial dependence. As a result of all these somatic (pain, organ complications, movement limitations), psychological, social, occupational and therapeutic (including side effects of drugs) problems the patient is forced to work out their own methods of coping. Being able to accept the disease and its consequences can contribute to a significant improvement in the patient’s quality of life [8]. Acceptance of illness allows for identification of the affected person’s resources. Awareness of one’s own capabilities and limitations allows the patient to adjust actions according to individual capabilities [7].
The aim of this study was to assess the functioning of a patient suffering from granulomatosis with polyangiitis (GPA).
Case description
The study was conducted on a 48-year-old patient suffering from granulomatosis with polyangiitis. The study was carried out during the patient’s hospitalization in a specialized unit in Krakow in June 2022, with the permission of the management of the facility. The study was conducted in accordance with the principles of the Declaration of Helsinki. The patient gave his informed consent to participate in the study. The study employed a customized interview questionnaire and standardized survey instruments, including the Functioning in Chronic Illness Scale (FCIS) by A. Kubica [9;10]; Acceptance of Illness Scale (AIS) by B. J. Felton et al. adapted by Z. Juczyński; and the Satisfaction with Life Scale (SWLS) by E. Diener et al. adapted by Z. Juczyński [11].
During the examination, the patient remained in logical verbal contact, declared a positive attitude towards his current condition, hospitalization, and life situation from before hospitalization. The patient had been hospitalized several times in the past. In addition, he suffered from chronic bronchial asthma and hypertension, and his BMI score was indicative of first-degree obesity. At the same time, the patient was characterized by abnormal physical exercise intolerance, shortness of breath and cough with expectoration of sputum. The patient’s medical history indicated complaints of joint pain. He did not report significant complaints regarding other systems. The patient denied having any addictions and allergies. He was professionally active and described his working conditions as good. He mentioned the lack of leisure time. The patient lived with his family
The Functioning in Chronic Illness Scale (FCIS) used in the study allowed for the assessment of the respondent’s functioning in general, as well as on three subscales: Impact of the disease on the patient, Impact of the patient on the disease and Impact of the disease on the patient’s attitudes [9;10]. As interpreted, an overall scale score of ≤78 points indicates a low level of functioning in chronic disease, and a score of ≥94 points indicates a high level. A range of 79-93 points indicates a medium level. For the subscale Impact of the disease on the patient, the medium range is between 24-33 points; the low level ≤23 points, and the high level ≥34 points. For the subscale Impact of the patient on the disease, the medium level ranges between 25-29 points; a low level ≤24 points; a high level ≥30 points. In the last subscale Impact of the disease on the patient’s attitudes, a range of 28-33 points indicates a medium level, a score of ≤27 points indicates a low level, and a score of ≥34 points indicates a high level [9;10].
The examined patient’s score on the Functioning in Chronic Illness Scale (FCIS) was 83 (the maximum score on the scale is 120). In the case of the respondent, the overall score for functioning in illness was within the average level (79-93 points). In the detailed analysis, scores from the three subscales mentioned above were also included. Detailed data are presented in Table 1.
Table 1
Functioning of the examined patient in chronic disease - FCIS results
On the subscale of Impact of the disease on the patient, the respondent’s score was on the average level. The respondent indicated that his physical performance was definitely not like before the disease; at the same time, he declared that the disease did not limit his physical activity or social and family contacts.
On the subscale of Impact of the patient on the disease, the respondent’s score was the lowest. Such a score may indicate that the respondent felt he did not have a big influence over the course of the disease. He strongly disagreed with the statement that his illness could be the result of his negligence. He stressed that his well-being in the future would depend on him and taking medication regularly could improve his health. According to the patient, daily physical activity cannot improve his health and because of his illness he should avoid even small efforts.
On the last subscale, which was Impact of the disease on the patient’s attitudes, the respondent’s score was also on the average level and was related to his attitudes in the situation he was experiencing. It is noteworthy that when facing illness, the patient decided to take active care of his health. At the same time, he felt helpless in face of the disease, although he believed that his health could still improve. He was unlikely to think that he would feel worse as a result of the disease, and definitely did not feel that he had lost everything because of his condition. The respondent emphasized that he definitely thinks positively about the future.
Acceptance of the disease is an equally important aspect in the context of functioning with a chronic disease. On the Illness Acceptance Scale, the respondent scored 32 points (range of 8-40 points). A detailed analysis showed that the patient had no problems adapting to the limitations imposed by the disease, was not more dependent on other people, did not have lowered self-esteem related to his health situation, and felt self-sufficient and needed. However, the respondent’s medical condition caused limitations in the activities he enjoyed most. He did not explicitly state that because of his illness he was a burden for his family, or that people staying with him might feel embarrassed.
The examined patient was also asked to assess his satisfaction with life according to the SWLS scale. The respondent indicated to what extent he agreed with each statement on the scale, which translated into an overall score. He scored 26 points on the aforementioned scale (range of 5-35 points). Following the rules for interpreting the SWLS scale, the patient’s raw score was converted to sten values. His score corresponded to sten 7. It was concluded that the patient under study presented a high degree of satisfaction with his life (sten 7-10).
Discussion
Granulomatosis with polyangiitis (GPA) is a rare condition belonging to a group of autoimmune diseases [12]. However, due to its chronic, recurrent nature [3], varied clinical picture [2] and serious potential complications following the medications used [3, 13], it is becoming an important clinical problem, also in terms of the patient’s functioning in the disease. In patients with GPA, lesions can involve multiple systems and organs, and the clinical manifestations that appear in patients depend on the location of the lesions and can affect patients’ daily life [14]. This study presents a case study of a patient hospitalized for granulomatosis with polyangiitis. An analysis of the subject’s somatic condition identified symptoms of the disease, which mainly involved the respiratory system. It should be noted that the clinical picture may also have been influenced by symptoms of other comorbidities. Analyzing the respondent’s results, it was concluded that the overall score of the Functioning in Chronic Illness Scale (FCIS) was within the average level. Also a similar level was reached for the score of the subscale of Impact of the disease on the patient and Impact of the disease on the patient’s attitudes. On the other hand, on the subscale of Impact of the patient on the disease, the respondent had the lowest score. On the whole, it can be said that the respondent’s functioning in chronic illness was on an average level. It should be remembered that patients can lead a normal lifestyle, of course, taking into account the type of work and possible risks. However, this will be possible once the disease is in remission, and patients are on maintenance medication [14].
In terms of the disease, patients’ subjective experiences appear to be crucial, and these may differ from doctors’ assessments. Therefore, patients’ perspectives should be taken into account when evaluating treatment outcomes [15]. Herlyn et al. conducted an analysis of self-reported disease burden in patients suffering from vasculitis. This study seems interesting because the results obtained from patients of different nationalities were compared. In the analysis, a significant percentage of patients reported a disease remission or its mild course. It was reflected in the patients’ assessment of their disease symptoms. According to Herlyn et al. fatigue and loss of energy were considered the most important disease burdens, while symptoms related to organ damage were rated lower. In addition to those previously mentioned, weight gain, joint pain and sinusitis appeared in the ranking of symptoms.
In our study, the patient also experienced clinical symptoms, including abnormal exercise intolerance, shortness of breath, cough, and a medical history indicating joint pain.
The evidence from the scientific literature suggests that the clinical course of the disease significantly affects the overall quality of patients’ lives, as they become more susceptible to depression, anxiety and may experience sleep disturbances, mood disorders, neurocognitive changes and may be unable to work [16,17]. According to a study by Cazzador et al, the quality of life of patients suffering from antineutrophil cytoplasmic antibodies-associated vasculitis (AAV) is significantly reduced, especially in the case of its laryngological manifestations. This impact is particularly significant in terms of social functioning as it leads to limitations in patients’ both everyday routine and professional activities [16]. Therefore, it seems important to assess the impact of the disease on patients’ quality of life and their ability to work. Similarly, the duration of the disease is not insignificant. Benarous et al. analyzed these aspects in detail and showed that the quality of life of patients with AAV was impaired compared to the general population. This was especially true for patients with eosinophilic granulomatosis with polyangiitis (EGPA) and for those suffering from a long-term disease, although GPA patients accounted for 67% of those studied, EGPA ones for 21%, and those with microscopic polyangiitis (MPA) for 12%. This study shows that most patients in the <60 year group were still working. Among those surveyed by Benarous et al, 23% of those working felt that the disease limited the nature of their work in qualitative terms, and 43% felt that the disease limited the amount of work they could do [18]. Referring to the results of our own study, it is noteworthy that the patient was professionally active despite his chronic disease. He declared that his physical capacity had decreased compared to the period before the disease, however, the disease did not reduce his physical activity. Unfortunately, the patient’s medical condition resulted in limitations in the activities he enjoyed most. Although he did not receive the maximum number of points on the AIS scale, a trend towards acceptance of the disease could be observed. This is important because the consequences of chronic diseases are complex and relate to different spheres of patients’ functioning. When faced with a chronic disease, patients may change their previous priorities and may either accept their health condition or not. Acceptance of the disease and support from the loved ones and from medical staff can significantly improve the quality of life. People who accept the disease are less likely to experience negative emotions [19]. In a study by Brzozowska et al. the level of disease acceptance was higher in a group of patients with systemic lupus erythematosus or inflammatory bowel disease compared to patients with rheumatoid arthritis (RA). In a study by Barańska et al. also in a group of RA patients, the AIS scale results indicated that one in two respondents presented a medium level of acceptance of the disease, and one in four presented no or low adjustment to the disease [20]. According to Hamerlińska et al. in the assessment of acceptance of the disease it becomes more and more important to be as little dependent on family and friends as possible and to cope with the limitations caused by the disease. At the same time, the author emphasized that the higher the level of acceptance of a rare disease, the higher the level of satisfaction with life among the respondents [21]. A multicenter study by Kotulska et al. showed the complexity of the problem of patients’ satisfaction or dissatisfaction during biological treatment and suggested that further research should be conducted to examine specific aspects of the problem. Kotulska et al. found that satisfaction, especially in the case of chronic diseases, has an impact on patients’ compliance with medical recommendations. Hence, it seems essential to measure patients’ satisfaction in the process of medical care [22]. The assessment of satisfaction with life is the outcome of comparing one’s own situation with self-established standards, and if the result of the comparison is satisfactory, it produces a feeling of satisfaction [11]. This is how the patient surveyed assessed his satisfaction with life. The score obtained by the respondent indicated a high degree of satisfaction with life and was higher than the scores in the SWLS standardization group among men (20.11 points) in Juczyński’s study [11]. In conclusion, it should be emphasized that in the assessment of patients, attention should be paid not only to the physical aspect, but also to the psychosocial aspect of the disease.
Conclusions
In the case of the examined patient the value of the overall score for functioning in chronic disease was on an average level. Also, the respondent’s functioning in terms of the impact of the disease on the patient and on the patient’s attitudes was proved to be on a similar level.
It was concluded that the respondent did not have full control over the course of the disease, which was not the result of his negligence. At the same time, he showed a tendency to accept his condition.
Despite some limitations associated with functioning in chronic disease, the patient presented a high degree of satisfaction with life.
In clinical practice, it is essential to assess the patient’s functioning in a chronic disease. Identification of deficits in physical and psychological functioning of the patient in a chronic disease requires implementation of appropriate measures by the therapeutic team.
