1. SLE Management

SLE can cause widespread inflammation and damage to multiple organs (joints, tendons, skin, heart, lungs, brain, kidneys, and blood vessels). Currently, the reported prevalence of SLE in the United States is 20~150 cases per 100,000 [4, 5]. The average 10-year survival rate exceeds 90%, and the rate drops to 80% for the 15-year survival rate [6]. Patients with SLE may experience major symptoms, including fatigue, skin rashes, fever, and joint pain. Other complications include sun sensitivity, oral ulcers, arthritis, pleuritis, serositis, kidney disease, seizures, psychosis, and blood cell and immunological abnormalities [1, 2, 3]. With a pattern of fluctuating disease activity, some patients may experience intermittent episodes of active disease (flare-ups) followed by periods of low or absent disease activity (remission) [1, 2, 3]. Due to multi-organ involvement, SLE treatment often involves a multidisciplinary team approach (i.e., rheumatology, nephrology, cardiology, and dermatology) [1, 2, 3].

Furthermore, patients with SLE face numerous physical, psychological, and social challenges during their illness [7, 8]. Meanwhile, a considerable proportion of patients do not have sufficient knowledge of their disease, sequelae, or the therapeutic course. As a consequence, poor medication education has a negative impact on the clinical outcomes of patients [9]. Research has also shown that psycho-educational intervention programs are successful in improving self-efficacy and lowering patients’ mental burden and fatigue [8]. However, approximately 75% of patients with SLE report unfulfilled educational needs [10].

2. Patient Education in Information Value Chain

Education designed for patients with SLE is often recommended by field experts [11], however, such recommendations provide ambiguous instructions regarding essential contents and timing. The delivery of inappropriate information at improper care stages may increase anxiety levels in individual patients. If patients are given too much information too soon, the evidence suggests that the incidence of depression can increase among them [11, 12]. Patients also acknowledged that needs vary according to individuals and situations, depending on their responses to disease progression. Significantly, Brown [13] notes that SLE can lead to significant worry and distress, especially during delays in diagnosis and times of high disease activity. Thus, it is recommended that providing appropriate individualized information to people with SLE at the right care stage enables them to use self-management tools to gain control over everyday symptoms [13]. However, patient education research has rarely focused on different care stages with relevance to educational content.

Chronic care management with relevance to patient education is complex as it involves longitudinal disease progression and management. Originating from a business model, Porter and Teisberg [14] argued that patient education should be included at each stage of chronic care, ranging from prevention (or screening), diagnosis, and intervention to monitoring (or managing) [15, 16, 17]. Although not traditionally handled by clinicians, involvement in prevention and monitoring activities, such as self-screening at an early stage prior to a confirmed diagnosis, can help raise awareness. Diagnosis aids in early disease detection, which often helps in reducing the impact and severity of a disease. Successful treatment relies on patients receiving proper information in the preparational stage for specific procedures and therapies. Intervention, whether with drugs or procedures, should be performed with the most suitable specialists and technologies. Finally, patient care must be monitored and managed to continually learn and improve as well as follow-up on treatment adherence. For the abovementioned reasons, Porter’s model can support the development of a strategy for multi-stage patient education that is rarely addressed in current practices.

Porter's value chain analysis is based on the collection of operations employed by a company in a certain industry to generate market value. Applied to information-intense industries, such as healthcare, the chain starts with an interaction in which data is provided to make informed clinical decisions. Thus, the decisions influence the course of care and, in turn, patient outcomes. Therefore, information has value only if it is expected to result in changes [15]. A six-stage information value chain that connects the initial encounter with information to the ultimate effects of information is recommended in the healthcare context [15]. As changes in one stage of a value chain may or may not affect the succeeding stages, the information value chain provides a prism to examine and evaluate each stage separately. Importantly, whether the information could impact a patient's self-care process or providers’ clinical workload, the value chain can be modified in the framework. For instance, our analysis highlights the provider’s viewpoint, so the first three clinical stages (screening, diagnosing, and preparing) were emphasized, while the last stage (monitoring) can be further separated into sub-value stages such as recovering/rehabilitating, and monitoring and managing.

Using Porter’s value chain analysis as a theoretical framework, this study aimed to identify and examine the available research concerning or related to the information needs of patients with SLE focusing on four primary activities of the Chronic Disease Value Chain (CDVC): screening, diagnosing, intervening, and monitoring. This study addresses three research questions: (1) What are the essential contents and timing of delivery of education materials regarding patients with SLE identified by the literature review? (2) What are the essential contents of the education material regarding patients with SLE recognized in the clinical practice guidelines? (3) What are the key concepts extracted from education materials regarding patients with SLE?

1. Data Sources

Our study samples consisted of three analytic sources: (1) scientific journal articles extracted from the PubMed database, (2) clinical practice guidelines based on the 2019 European League Against Rheumatism (EULAR) and British Society for Rheumatology Standards for SLE, and (3) online patient education materials for SLE [18, 19]. First, a PubMed literature database search was performed using a combination of Medical Subject Headings (MeSH): "patient education as topic" [MeSH terms] OR "patient education" [MeSH terms] AND "lupus erythematosus, systemic" [MeSH major topic]. This initial search yielded 115 papers in English, and manual reviews were performed to include only relevant articles pertaining to the essential components of SLE knowledge in the patient education context. After removing non-relevant papers (i.e., clinician target, medical education, editorial, and opinion), we identified 14 papers for valid analysis. Figure 1 shows the details of the literature selection in a flow diagram.

Figure 1
Flow diagram of PubMed article selection process.

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Second, we carefully reviewed the clinical practice guidelines recommended by one of the leading professional rheumatology associations designated for SLE, EULAR. According to the Institute of Medicine, clinical practice guidelines are not only for clinicians but also for patients to assist in their decisions toward appropriate healthcare for specific clinical circumstances [20]. EULAR [18] was used to identify essential content using four value stages: screening, diagnosing, intervening, and monitoring. Primary and secondary information for use in education regarding patients with SLE was identified in each value chain. These guidelines are intended to offer concise instructions on how to provide healthcare services. Therefore, we adopted these to extract patient education content according to the four value stages.

Third, we extracted key concepts from 10 major patient education resources: the American College of Rheumatology, National Institute of Arthritis and Musculoskeletal and Skin Diseases, Center for Disease Control and Prevention, WebMD, MedlinePlus, UpToDate, Mayo Clinic, Merck, Wikipedia, and Medscape. Only public access data were used in this study; therefore, the study did not require IRB review.

2. Four Value Chain Stages as Study Measures

We operationalized two analytic measures: essential content and key concepts. The essential content refers to what was recommended or indicated as essential SLE knowledge in the medical literature and clinical practice guidelines within the education context regarding patients with SLE. Waldron et al. [11] reported that patients in their study indicated that they needed scientific explanations to overview SLE in general at the time of diagnosis, whereas they expressed the need for symptom control and treatment management information as their therapeutic course was determined. In this example, we identified “general disease information”, “therapeutic management”, and “symptom control” as essential contents, and “at the time of diagnosis” and “during the course of therapy” as delivery timing. As regards, Essential content was used to build an information typology for use in education about patients with SLE.

Additionally, essential content refers to the education that is imparted in different stages of the information value chain. Modified from Porter’s framework, we first identified the information recommended by clinical practice guidelines and grouped them into four value chains: “screening, diagnosing, intervening, and monitoring”. Screening is the stage in which information is valued prior to a clinical visit. In this category, we focused on identifying patients’ awareness of SLE-specific symptoms for further pursuant to clinic visits. Diagnosis is performed by clinicians, and patients are clearly informed about their apparent signs and symptoms, which are linked to tests for the final decision. In this context, essential content is used to educate patients about the diagnostic process. Intervening refers to any therapeutic regimens and course-related context involving efficacy, side effects, and compliance. Monitoring follows the management or adherence to a planned therapeutic course.

In this study, the essential content identified from both medical literature and clinical practice guidelines was mapped with patient education materials. A collection of online patient education materials (n=10) was manually annotated by three authors (JK, SK, and MR), identifying the key concepts and value chain stage each belonged to. Once key phrases or words were identified (and agreed to by the three authors), we ran a clinical natural language processing software, “Clinical Language Annotation, Modeling, and Processing Toolkit,” developed by the University of Texas. This process is essential for identifying clinical concepts, rather than simple keywords. We used the unified medical language system’s concept unique identifier, which uniquely represents a single meaning, and the concept name was chosen as a word to represent the whole concept. To be considered “key,” a concept, as identified by the concept unique identifier, needed to appear more than three times across all patient education materials. As a concept could appear in more than one stage of the value chain depending on the context, concepts needed to meet the threshold per value chain stage for inclusion as a key concept. For instance, a concept identified as screening four times but diagnosing only once in all patient education materials would only be considered a key concept for the screening stage of care. For follow-up studies by others, the following concepts and keywords extracted in tables could be used to populate what to highlight in each stage of SLE care management.

1. SLE Essential Contents from Medical Literature Review

Of the 14 studies, six were based in the USA and one each in Finland, Germany, Iran, the Netherlands, Sweden, Taiwan, and the United Kingdom. The objective of the studies was to examine the effectiveness of the intervention in providing information about specific topics related to SLE. Specific topics included an ethnic-specific cholesterol-lowering diet [21], self-management behavior [22], body image management [23], steroid pulse therapy [24], and immunosuppressive drugs [25, 26]. Four studies examined the effectiveness of an intervention to provide comprehensive SLE information regarding patients’ knowledge [3, 8, 27, 28]. Furthermore, one study described patients’ educational needs for SLE [2], and another study surveyed the efficacy of the information policy given to patients with SLE [29]. Additionally, psychoeducational interventions, such as basic cognitive strategies, have been evaluated for cognitive dysfunction in patients [30]. Table 1 presents the conclusions of the medical literature analysis.

Table 1
Essential Components of Systemic Lupus Erythematosus Patient Education in Medical Literature

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We further analyzed the essential content using the four value chain stages. First, five of the selected studies provided information regarding SLE screening. These include a general understanding of disease manifestations such as SLE definition, pathophysiology, etiology, prognosis, and progress [2, 8, 24, 28], signs and symptoms [8, 27, 31], risk factors [8, 27], and exacerbating factors [8]. Second, the diagnosis value chain includes three studies highlighting SLE information from laboratory tests [3, 24] and assessments [28]. Third, SLE information related to the intervening value stage contains drug therapy options [2, 24, 25, 26, 27, 29], compliance and drug administration [8, 22], side effects [24, 25, 26, 28], pain management [2], and efficacy and safety of pharmacological substances [28].

In the end, the monitoring value chain contains a wealth of behavioral adjustments and recommendations. This value chain highlights SLE information about the prevention and management of disease complications [3, 8, 22, 24, 27, 28], patient support systems [2, 3, 27, 28], psychosocial support [2, 8, 28, 30], diet restrictions [3, 8, 21, 24], daily activities [8, 24, 28, 29], pregnancy related care, including breastfeeding and fertility [3, 25, 26, 27], skin care and cosmetics [3, 8, 23], regular check-ups [8, 22], smoking cessation [29], sun protection behavior [29], and use of complementary and alternative medicine [29]. In our analysis, the monitoring stage included most of the information recommended by the medical literature in Table 1.

2. SLE Essential Contents in Clinical Practice Guidelines

The information included in the guidelines is based on the criteria of the American College of Rheumatology or Systemic Lupus International Collaborating Clinics, most often used by health providers [18]. In the screening stage, the guidelines imply that patients with SLE or primary care physicians may need to know the initial signs and symptoms, risk factors, and triggers, so that they can promptly seek professional help or be referred to rheumatologists. Signs and symptoms include fatigue, weight loss, fever without focal infection, arthralgia, myalgia, malar rash, photosensitivity, pleuritic chest pain, new-onset Raynaud’s phenomenon, and mouth sores. Risk factors also include being a woman of African, South Asian, or Chinese descent [19]. SLE can also be triggered by sunlight, concurrent or recurrent infections, hormonal changes, and the timing of previous disease-related therapeutic changes.

In the diagnosis stage, clinical or laboratory findings regarding activities such as specifying, ordering, and testing based on the American College of Rheumatology criteria were noted as information to be discussed with the patients. This process includes clinician assessment of malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, serositis, renal disorders (protein urea, cellular case, red cell, hemoglobin, etc.), neurological disorders (seizures, psychosis), hematologic disorders (hemolytic anemia with reticulosis, leukopenia <4,000, lymphopenia <1,500, thrombopenia <100,000), immunological disorders (anti-dsDNA, anti-Smith antibody, antiphospholipid antibodies, abnormal serum levels of immunoglobulin g, immunoglobulin m), and results of tests for Antinuclear Antibodies (ANA). Systemic Lupus International Collaborating Clinics [19] criteria were also described, including clinical and immunologic criteria, besides biopsy-proven nephritis compatible with SLE in the presence of ANA or anti-dsDNA antibody. Although the initial screening and diagnosis stages refer to similar assessments of early signs of clinical symptoms, they are slightly different in utilizing either providers or patients’ viewpoints, so they are somewhat repetitively stated. While screening is more focused on non-rheumatic specialists, such as general practitioners or potential patients with SLE, the later diagnosis stage includes laboratory confirmation procedures that require a rheumatologist’s specialty.

In the intervention stage, patients are supposed to be apprised of the aims of the treatment, options for drug therapy, and side effects of drug therapy. These include major medications such as hydroxychloroquine, glucocorticoids, immunosuppressive therapy (i.e., methotrexate, mycophenolate mofetil, azathioprine, and cyclophosphamide), biologics (i.e., belimumab, rituximab), and nonsteroidal anti-inflammatory drugs. They must also be informed of the side effects of individual drugs such as myelosuppression, hepatotoxicity, lymphoproliferative disorders due to azathioprine, and serious infections and malignancies due to belimumab. Notably, the intervening stage highlights information about care providers, including primary care providers who do not usually treat major organ systems; rheumatologists for assessing disease activity, complications, and adverse effects; and family physicians to monitor medication effects with relevance to disease activity.

In the monitoring stage, patients must manage SLE to avoid disease flares, drug complications, and comorbidity management with relevance to lifestyle modifications. At this stage, information is necessary regarding complications, the meaning of clinical and laboratory deterioration, ways of promoting appropriate risk reduction strategies, and additional community and social services, if needed. The details are presented in Table 2.

Table 2
Systemic Lupus Erythematosus Essential Contents Identified from Clinical Practice Guideline

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3. SLE Key Concepts Mapped into Unified Modeling Language System (UMLS) Dictionaries

The natural language processing model created for this study identified 1,144 unique concept identifiers in the patient education materials. The most commonly occurring concepts were “butterfly rash”(C0277942) and “anti-inflammatory drugs”(C0003211), each extracted 20 times. Interestingly, these appeared more frequently than “SLE” (C0024141), the concept for the disease itself, which was extracted 19 times. This was closely followed by “antinuclear antibodies”(C0151480), which was extracted 18 times.

The model classified each concept according to the value stage for which the information provided would be the most appropriate. Concepts were most frequently classified as screening (n=646). Monitoring had far fewer concepts (n=459), while diagnosing (n=379) and intervening (n=377) showed the least. Although a concept could be included in more than one stage, depending on the context, the model was consistent in allocating key concepts. For example, the concept of "Alopecia (hair loss)" (C0002170) has been identified 14 times in patient education materials. It was classified as screening for all but one, where it was mentioned in diagnosis. Hair loss, clinically known as alopecia, is one of the diagnostic criteria for SLE. However, in patient education materials, it is commonly referred to as a sign of the disease that patients should be aware of, thus making the model’s classifications accurate.

After filtering the results to concepts that appeared more than three times in a value stage, the 66 concepts shown in Table 3 were identified as key concepts across the four stages of the value chain. Screening retained the most concepts and highlighted the common signs and symptoms believed to be indicative of SLE along with demographic risk factors. Of the last three stages, diagnosis retained the most concepts. The refined intervening concepts emphasized the drugs used for the treatment of SLE and their beneficial effects. Monitoring resulted in the largest reduction in concepts. This reflects the lack of consistent information presented in the educational materials at this stage. This stage produced the most generic concepts. While the identified key concepts are still ambiguous, they represent some of the most specific advice given for long-term disease management (i.e., healthy diet and avoiding sun exposure).

Table 3
Systemic Lupus Erythematosus Key Concepts Mapped from Patient Education Materials

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1. Practical Implication and Limitation

The SLE information value chain was developed using a collection of research concerning the information requirements of patients with SLE and best practices for treatment and self-management. For several reasons, healthcare providers do not always implement best practices. The repetition of topics throughout the value chain indicates that information might best be given multiple times in the care cycle. It is likely that patients might need slightly different information about the same topic depending on the care stage. However, patients are usually given only one worksheet of basic SLE information when first diagnosed with the condition. Research routinely shows that patients do not recall receiving educational information, and our study demonstrates that many concepts related to necessary information are not readily available in Patient Education Materials (PEMs) or patient notes. This study highlights key SLE concepts that contribute to the reinforcement of SLE patient education at different stages.

Some of the information identified in the SLE value chain is not included in the PEMs or clinical practice guidelines that we studied, nor are the patients told the information by anyone on their healthcare team. However, we limited our analysis to only two major clinical practice guidelines (European and North American), which might misrepresent some important details of the SLE practices and guidelines in Asian or other countries. Future studies should include guidelines that can add value to diverse racial and ethnic population-based clinical findings.