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Selected Immune Disorders and Disability (2022)

Chapter: 1 Introduction

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Suggested Citation:"1 Introduction." National Academies of Sciences, Engineering, and Medicine. 2022. Selected Immune Disorders and Disability. Washington, DC: The National Academies Press. doi: 10.17226/26595.
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1

Introduction

The Social Security Administration (SSA) has requested that the National Academies of Sciences, Engineering, and Medicine (NASEM) assemble a committee to review selected conditions related to the immune system. This report responds to that request. In particular, SSA is interested in the current status of the diagnosis, treatment, and prognosis of immune system disorders including systemic lupus erythematosus (SLE), scleroderma, polymyositis, Sjögren’s syndrome/disease, and inflammatory arthritis.

SSA provided the committee with the following Statement of Task.

STATEMENT OF TASK

An ad hoc committee of the National Academies of Sciences, Engineering, and Medicine will review selected conditions related to the immune system and produce a report addressing the current status of the diagnosis, treatment, and prognosis of those conditions based on published evidence (to the extent possible) and professional judgment (where evidence is lacking):

  1. Provide an overview of the current status of the diagnosis, treatment, and prognosis of select immune system disorders, including systemic lupus erythematosus, scleroderma, polymyositis, Sjögren’s syndrome, and inflammatory arthritis, but excluding HIV, in the U.S. population and the relative levels of functional limitation typically associated with the immune system disorders, common treatments, and other considerations.
Suggested Citation:"1 Introduction." National Academies of Sciences, Engineering, and Medicine. 2022. Selected Immune Disorders and Disability. Washington, DC: The National Academies Press. doi: 10.17226/26595.
×
  1. For the immune system disorders identified in task 1, describe to the degree possible:
    1. The average age of onset;
    2. The professionally accepted diagnostic techniques used in identifying immune system disorders (for example, laboratory and clinical findings) and how the techniques differ for adults and children (if applicable);
    3. The methods for differentiating clinical severity (for example, classifiers such as “moderate” or “severe”), how the methods are determined (for example, by specific laboratory findings), and what the methods mean in terms of treatment, prognosis, and functional limitation;
    4. The usual course of the disorder, including any differences in the course of the disorder for adults and children (if appropriate);
    5. The likelihood, frequency, and duration of changes in the severity of symptoms such as flare-ups or remissions (if appropriate);
    6. The possibility and likelihood of reducing the severity of symptoms (if appropriate), and the treatments or circumstances that lead to marked improvement; and
    7. Secondary impairments that result from either the immune system disorder or the treatment (if appropriate).
  2. For the immune system disorders identified in task 1, identify the types of treatments available and describe to the degree possible:
    1. The clinical practice guidelines for receiving the treatments;
    2. The settings in which the treatments are provided;
    3. What receipt of the treatments indicates about the severity of the medical condition;
    4. The likelihood of improvement when receiving the treatments and the period over which the improvement would be expected; and
    5. Any limitations on the availability of the treatments (other than due to financial circumstances or the patient’s election), such as whether treatments are considered experimental, remain in the trial phase, or are only available in certain geographic areas.
  3. For the immune system disorders identified in task 1, provide a summary of select treatments currently being studied in clinical trials.
  4. For the immune system disorders identified in task 1, identify to the degree possible the functional limitations associated with each disorder, including physical functioning limitations, mental functioning limitations, limitations resulting from common treatments, and variations in functioning (for example, during flare-ups vs. remission), and how such limitations would present in a typical medical record.
Suggested Citation:"1 Introduction." National Academies of Sciences, Engineering, and Medicine. 2022. Selected Immune Disorders and Disability. Washington, DC: The National Academies Press. doi: 10.17226/26595.
×

In responding to the Statement of Task, the committee introduces background information related to immune disorders, followed by the concepts of disability and functional status. The committee then discusses the disability evaluation process that SSA employs and turns to the measurement of those concepts in the specific context of rheumatology. The chapter ends by explaining the committee’s approach to the task and the organization of the report.

IMMUNE DISORDERS

Immune disorders contribute substantially to morbidity, mortality, and health care costs in the United States. They are the most common cause of morbidity in women in the United States and one of the top 10 causes of death in women under the age of 65. More than 50 million Americans are living with an immune disorder. It is estimated that more than 100 billion health care dollars are spent in the United States each year in the management of autoimmune patients (Rosenblum et al., 2012). A recent review article (Varadé et al., 2021) reported that more than 100 autoimmune disorders have been identified. Although those diseases were historically considered to be rare, epidemiological data have shown that they affect approximately 3–5 percent of the population worldwide. Some of the most common autoimmune disorders are type 1 diabetes, rheumatoid arthritis (RA), SLE, and inflammatory bowel disease. Although significant progress has been made in understanding the mechanisms of autoimmune disorders

Suggested Citation:"1 Introduction." National Academies of Sciences, Engineering, and Medicine. 2022. Selected Immune Disorders and Disability. Washington, DC: The National Academies Press. doi: 10.17226/26595.
×

and the nature of self-tolerance, those disorders remain major burdens on health systems around the world. Further, the committee notes that with new findings in fundamental immunology, the nomenclature for many of the diseases of the immune system continues to evolve.

Throughout this report, several different terms are used to characterize the group of diseases that are its focus. There are slight differences among the terms. The terms “rheumatic disease” and “rheumatologic disease” are interchangeable and refer to conditions that are caused by chronic inflammation of one or more of the connective tissues; these conditions are usually the result of a disordered immune system. “Autoimmune disorders” are caused by an immune response against self-antigens and are characterized by the presence of autoantibodies. As previously mentioned, there are more than 100 autoimmune disorders, and multiple organ systems can be affected, with rheumatic diseases such as SLE and RA forming a subset of these conditions. “Immune disorders” include autoimmune disorders, but also include immunodeficiency disorders, which may be hereditary or acquired and are beyond the scope of this report. Diseases that are diagnosed in childhood may resemble diseases that are diagnosed in adulthood but may have different phenotypes. Those diseases, known as “juvenile” or “childhood-onset” diseases, continue into adulthood, yet are still referred to as “juvenile” disease in adults. The terms used in the report are those that are used in the references cited, in an effort to accurately reflect the information in the source material. Additionally, the committee notes that in the disease-specific chapters, mentions of “severity” refer to clinical severity, rather than SSA’s program definition.

CONCEPTUALIZING DISABILITY

According to SSA, the statutory definition of “disability” for adults is the “inability to engage in any substantial gainful activity by reason of any medically determinable physical or mental impairment which can be expected to result in death or which has lasted or can be expected to last for a continuous period of not less than 12 months.”1 Substantial gainful activity is defined via an earnings threshold. In short, for an adult to be deemed disabled, a medical condition must lead to limitations that themselves affect the ability to earn in the labor market. A finding of disability in both adults and children depends on the severity of functional limitations arising from the claimant’s impairment or combination of impairments.

Recent National Academies reports have provided a detailed background of the evolution of concepts of disability over the past several

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1 42 U.S.C. § 423(d)(1) and 42 U.S.C. 416(i).

Suggested Citation:"1 Introduction." National Academies of Sciences, Engineering, and Medicine. 2022. Selected Immune Disorders and Disability. Washington, DC: The National Academies Press. doi: 10.17226/26595.
×

decades (NASEM, 2019, 2020). One relevant conclusion from those reports is the growing recognition that the effects of a given medical condition on functioning, activity, and participation are mediated by an individual’s physical and social environments. This recognition has been embodied in the International Classification of Functioning, Disability and Health (ICF) developed by the World Health Organization (WHO) and is illustrated in Figure 1-1.

The diagram in Figure 1-1 depicts a framework describing the interactions among health conditions, body functions and structure, activity, and participation, all of which are elements of the conceptualization of disability, and all of which are mediated by contextual factors including environmental and personal factors. Note that the arrows in the diagram are always double-pointed, since the relationships can involve feedback. Similarly, the immune conditions discussed throughout this report and their impact on functioning and disability can be strongly mediated by contextual factors and environmental factors in particular, including cold environments or direct sunlight.

To illustrate how the ICF framework can be used to inform the process of disability onset and persistence in the case of a particular immune disorder, take the example of scleroderma. The work disability literature for scleroderma describes the importance of workplace accommodations (e.g., flexibility in number and scheduling of hours worked), environmental

Image
FIGURE 1-1 International Classification of Functioning, Disability and Health Framework.
SOURCE: WHO, 2002, with permission.
Suggested Citation:"1 Introduction." National Academies of Sciences, Engineering, and Medicine. 2022. Selected Immune Disorders and Disability. Washington, DC: The National Academies Press. doi: 10.17226/26595.
×

factors (e.g., cold weather and Raynaud’s phenomenon), duration of the disease, demands at home, and the condition’s salience to employers, coworkers, and providers or referrals to occupational therapy (Lee et al., 2021) in mediating the relationship between the disease and work disability. Environmental factors (e.g., cold weather), participation restrictions (e.g., unreliable public transportation), and personal factors (e.g., home caregiving responsibilities), which are also referred to as “interrupters,” exacerbate a given condition’s impact on functioning and on earnings on a sustained basis (NASEM, 2019). Furthermore, focusing on the importance of contextual factors allows for the possibility of accommodations that might improve functioning or earnings capacity. For the immune disorders discussed in this report, it is important to consider the contextual factors, interrupters, and accommodations, and their effects on functional capacity, which can be self-reinforcing in both positive and negative dimensions. Next, the committee describes the details of how functional status in immune system disorders is evaluated in practice.

MEASURING FUNCTIONAL STATUS IN IMMUNE SYSTEM DISORDERS

Functional status may be assessed by self-report (questionnaires), performance-based measures (e.g., stair climbing, gait speed, grip strength), or proxy reports. Self-report measures capture the complex nature of functioning, incorporating “a person’s perception of their performance of tasks in their own environment and daily life, performance that could be potentially affected by many factors” (Coman and Richardson, 2006, p. 257). Self-reports may therefore be the most complete representation of the model described above, taking into account both the conditions themselves as well as contextual factors. Performance-based measures, in contrast, focus on a particular task under controlled, standardized situations but may lack a real-world context.

When self-report and performance-based measures are focused on the same construct, correlations tend to be moderate to high. In a review of studies, the strongest correlations were between self-reported mobility and observed community mobility (Coman and Richardson, 2006). However, self-reported measures often include questions about more complex behaviors, such as performing household chores, shopping, or self-care, which reduces the conceptual correspondence between performance measures and self-reported function. The performance of those complex behaviors may depend on the individual’s established adaptations, environment, or coping strategies and may vary over time depending on disease activity, pain, or energy levels (Coman and Richardson, 2006; NASEM, 2019; Terwee et al., 2006; Wilfong et al., 2020).

Suggested Citation:"1 Introduction." National Academies of Sciences, Engineering, and Medicine. 2022. Selected Immune Disorders and Disability. Washington, DC: The National Academies Press. doi: 10.17226/26595.
×

Since the 1980 introduction of the Health Assessment Questionnaire, a measure intended to measure function and disability in RA (Fries et al., 1980), rheumatology2 has relied heavily on patient-reported assessments of function. There are situations where impairments (e.g., in the range of motion) may be measured in clinical settings, and proxy measures may be collected for children. However, if functional status is assessed in clinical settings, it is almost exclusively done by patient-reported measures. Performance-based measures of functional status are primarily conducted in research settings.

Patient-reported measures of function are most likely to be available in medical records for people with RA, as well as for other diseases. The Centers for Medicare & Medicaid Services (CMS) incorporated the measurement of functional status into the Merit-Based Incentive Payment System (MIPS) program for RA, and the American College of Rheumatology recently recommended patient-reported functional status assessment measures for RA (Barber et al., 2019). Regular measurement of functional status in clinical settings is much less likely for other conditions. If functional status is measured, both disease-specific and generic measures may be used (e.g., Chang and Pope, 2020; Lane et al., 2020; Ogdie et al., 2020).

THE SOCIAL SECURITY ADMINISTRATION’S DISABILITY DETERMINATION PROCESS

When SSA evaluates a disability claim based on a physical or mental impairment, it requires sufficient evidence to: (1) establish the presence of a medically determinable physical or mental impairment or impairments; (2) assess the degree of functional limitation the impairment(s) imposes; and (3) establish the expected duration of the impairment(s). Once SSA establishes the presence of a severe medically determinable physical or mental impairment(s), it determines if the impairment(s) meets or medically equals (is equivalent in severity to) the criteria in the Listing of Impairments (Listings).3 The Listings are step 3 of the sequential evaluation processes for adults and children and serve as a “screen in” step for adults. For adults, the Listings describe, for each of the major body systems, impairments that SSA considers to be severe enough to prevent a person from doing any gainful activity, regardless of his or her age, education, or work experience—a

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2 Rheumatology includes a study of systemic autoimmune diseases including rheumatoid arthritis, systemic lupus erythematosus, Sjögren’s disease, scleroderma, polymyositis, and others.

3 The adult listings are available at http://www.ssa.gov/disability/professionals/bluebook/AdultListings.htm. The childhood listings are available at http://www.ssa.gov/disability/professionals/bluebook/ChildhoodListings.htm. Also see 20 Code of Federal Regulations (CFR) 404.1525, 404.1526, 416.925, and 416.926.

Suggested Citation:"1 Introduction." National Academies of Sciences, Engineering, and Medicine. 2022. Selected Immune Disorders and Disability. Washington, DC: The National Academies Press. doi: 10.17226/26595.
×

higher threshold than that required to establish disability in general. For children, the Listings describe, for each of the major body systems, impairments that SSA considers to be severe enough to cause marked and severe functional limitations in children. Immune system disorders that cause dysfunction in one or more components of the immune system are evaluated at step 3 of the sequential evaluation process under Listings 14.00 and 114.00, for adults and children, respectively. SSA organizes the discussions of immune system disorders into three categories: autoimmune disorders; immune deficiency disorders, excluding human immunodeficiency virus (HIV) infection; and HIV infection.

The committee notes that some of the introductory text of the Listings for immune system disorders refers to sources that do not incorporate all current knowledge, nomenclature, and classification criteria as defined by national professional organizations. For example, the Listings state that documentation of Sjögren’s Syndrome should generally be based on the most recent edition of the Primer on Rheumatic Diseases published by the Arthritis Foundation. However, the primer referenced is no longer in use, and clinicians now use criteria published by the American College of Rheumatology and International League of Associations for Rheumatology. The discussions in this report are based on the most current nomenclature and classification criteria.

For adults, if an impairment(s) is severe but does not meet or medically equal any Listing, SSA assesses the claimant’s residual functional capacity (RFC), the most an individual is able to do despite his or her functional limitations.4 SSA uses this RFC assessment to determine whether the claimant can do his or her past relevant work and, if necessary, to determine if the claimant can adjust to other work, in steps 4 and 5, respectively, of the sequential evaluation process.

When SSA determines whether the claimant’s impairment(s) is severe or meets or medically equals a Listing, or when SSA assesses the claimant’s RFC, it gathers information from the claimant, medical and nonmedical sources, and third parties. This includes information on the claimant’s impairment-related symptoms, such as pain, that may affect what he or she can do in a work setting.

For children, if the impairment(s) is severe, SSA assesses and makes a finding about whether the impairment(s) meets, medically equals, or functionally equals an item in the Listings. To do this, SSA evaluates the child’s functioning compared with children of the same age who do not have impairments.

SSA administers two federal disability programs: Social Security Disability Insurance (SSDI) and Supplemental Security Income (SSI). Although

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4 See 20 CFR 404.1545 and 416.945 and POMS DI 24510 Residual Functional Capacity (RFC).

Suggested Citation:"1 Introduction." National Academies of Sciences, Engineering, and Medicine. 2022. Selected Immune Disorders and Disability. Washington, DC: The National Academies Press. doi: 10.17226/26595.
×

the two programs have distinct nonmedical conditions for eligibility, SSA relies on the same disability determination process described above for both. The committee briefly describes the salient features of these two programs, since although they share the same disability determination process, they differ substantially with regard to nonmedical eligibility criteria and thus differ in terms of their beneficiary pools.

SSDI is part of the broader Old Age, Survivor, and Disability Insurance system and thus is a social insurance program: workers gain eligibility for retirement, disability, or auxiliary benefits via paying payroll taxes on earned income as required by the Federal Insurance Contributions Act. SSDI eligibility requires that workers be “insured” via a recent work requirement based on a sufficient number of quarters of coverage earned. Monthly benefits are then based on the indexed average of prior earnings. The details of eligibility and benefit calculation are available via SSA’s website,5 but the committee notes here that SSDI is designed for disabled individuals with a history of strong attachment to the labor force, with a benefit that increases the more a disabled individual has earned in his or her lifetime before disability onset. SSDI benefits can be withheld if beneficiaries consistently engage in earning at a substantial gainful activity level; however, other sources of income do not affect benefit receipt.

SSI is a categorical welfare program, with no work requirement, recent or otherwise, but with a means test that limits eligibility to individuals with $2,000 or less in assets. Furthermore, the receipt of earned income or income from other sources offsets SSI benefits. Due to its eligibility restrictions, SSI beneficiaries are more similar economically to the welfare program population than to SSDI beneficiaries, with substantially lower wealth and less historical attachment to the labor force (Burkhauser and Daly, 2007).

Although a substantial literature in disability policy has examined differences in SSDI and SSI applicants and beneficiaries, the subpopulation with the immune system disorders discussed in this report represents a fraction of the overall applicant and beneficiary population and has thus been of limited focus in prior studies. For example, data specific to immune system disorder claims are included in neither the SSA’s Annual Statistical Reports on SSDI nor the SSI Annual Statistical Report, where beneficiaries with immune system primary diagnoses are included in “Other” groupings. A 2015 Office of the Inspector General report on the Listings showed 18,922 initial adult allowances in 2013 under the immune system disorders body system.

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5 See https://www.ssa.gov/benefits/disability/qualify.html and https://www.ssa.gov/policy/docs/statcomps/di_asr/2019/background.html for eligibility requirements and benefit calculations (both accessed January 12, 2022).

Suggested Citation:"1 Introduction." National Academies of Sciences, Engineering, and Medicine. 2022. Selected Immune Disorders and Disability. Washington, DC: The National Academies Press. doi: 10.17226/26595.
×

More recently, the SSA provided the committee with information regarding the numbers of SSDI and SSI beneficiaries less than 65 years of age in the diagnostic categories relevant to this committee’s task (see Tables 1-1 and 1-2 below).

The committee notes that the diagnostic code for “inflammatory arthritis” accounts for 56–60 percent of all individuals receiving disability benefits for illnesses related to immune disorders. SSA identifies Sjögren’s syndrome, polymyositis and dermatomyositis, SLE, systemic sclerosis, and undifferentiated and mixed connective tissue disease under one diagnostic code, although they are distinct clinical entities, thus limiting the information available to the committee.

After Initial Disability Determination

The committee was unable to find information on the specific rates of award for SSDI or SSI applicants who allege disability due to an immune disorder. However, the committee notes that a majority of disability program applicants are denied benefits at initial application. Among SSDI claimants in 2018, only 36.6 percent were awarded benefits at the initial determination stage (SSA, 2019), and among adult SSI claimants, only 24.3 percent were awarded benefits at the initial determination stage (SSA, 2020).

Claimants can appeal those decisions within 60 days of an initial determination, and request a reconsideration. If that reconsideration results in denial, the claimant can then appeal for a hearing with an administrative law judge. If that hearing results in denial, the claimant can then request a review by the appeals council, and if that review results in denial, the applicant can file for an appeal through the federal courts system.

Furthermore, claimants who are awarded benefits are assigned a categorization based on the likelihood of medical improvement, which then determines when they will undergo their next medical CDR to determine if they remain medically eligible for disability benefits; the times when these CDRs take place are referred to as the claimants’ “diary dates.” Currently, there are two immune disorder conditions within the scope of the Statement of Task that explicitly fall under the “medical improvement not expected” criteria, which indicates that a CDR be scheduled no earlier than 5 years from adjudication. Those are:

  1. Diffuse Disease of Connecting Tissue: Listing 14.04—scleroderma or progressive systemic sclerosis,
  2. Spondylitis: Listing 14.09C—inflammatory arthritis (ankylosing spondylitis or other spondyloarthropathy, with ankylosis (fixation)
Suggested Citation:"1 Introduction." National Academies of Sciences, Engineering, and Medicine. 2022. Selected Immune Disorders and Disability. Washington, DC: The National Academies Press. doi: 10.17226/26595.
×

TABLE 1-1 Social Security Disability Insurance (SSDI) Beneficiaries Under Age 65, by Non-HIV Immune System Primary Diagnostic Group, December 2019

Diagnostic group Total Workers Widow(er)s Adult children
# % # % # % # %
All disabled workers
Ankylosing spondylitis or other spondyloarthropathies 7,119 3.5 6,865 3.6 120 2 134 3.9
Gout 5,326 2.7 5,240 2.7 74 1.2 12 0.3
Immune deficiency disorders, excluding HIV infection 6,431 3.2 6,120 3.2 109 1.8 202 5.9
Inflammatory arthritis 121,079 60.4 114,956 60.2 4,256 69.5 1,867 54.3
Sjögren’s syndrome, polymyositis and dermatomyositis, systemic lupus erythematosus, systemic sclerosis, and undifferentiated and mixed connective tissue disease 58,539 29.2 55,830 29.2 1,528 25 1,181 34.3
Systemic vasculitis 2,109 1.1 2,031 1.1 35 0.6 43 1.3
Total 200,603 100 191,042 100 6,122 100 3,439 100
Men
Ankylosing spondylitis or other spondyloarthropathies 4,699 9.5 4,587 9.4 15 7.5 97 10.3
Gout 4,631 9.3 4,592 9.5 (X) (X) (X) (X)
Immune deficiency disorders, excluding HIV infection 1,803 3.6 1,709 3.5 4 2 90 9.5
Inflammatory arthritis 30,930 62.2 30,256 62.3 129 64.2 545 57.7
Sjögren’s syndrome, polymyositis and dermatomyositis, systemic lupus erythematosus, systemic sclerosis, and undifferentiated and mixed connective tissue disease 6,877 13.8 6,667 13.7 21 10.4 189 20
Systemic vasculitis 761 1.5 745 1.5 (X) (X) (X) (X)
Subtotal 49,701 100 48,556 100 201 100 944 100
Suggested Citation:"1 Introduction." National Academies of Sciences, Engineering, and Medicine. 2022. Selected Immune Disorders and Disability. Washington, DC: The National Academies Press. doi: 10.17226/26595.
×
Diagnostic group Total Workers Widow(er)s Adult children
# % # % # % # %
Women
Ankylosing spondylitis or other spondyloarthropathies 2,420 1.6 2,278 1.6 105 1.8 37 1.5
Gout 695 0.5 648 0.5 (X) (X) (X) (X)
Immune deficiency disorders, excluding HIV infection 4,628 3.1 4,411 3.1 105 1.8 112 4.5
Inflammatory arthritis 90,149 59.7 84,700 59.4 4,127 69.7 1,322 53
Sjögren’s syndrome, polymyositis and dermatomyositis, systemic lupus erythematosus, systemic sclerosis, and undifferentiated and mixed connective tissue disease 51,662 34.2 49,163 34.5 1,507 25.5 992 39.8
Systemic vasculitis 1,348 0.9 1,286 0.9 (X) (X) (X) (X)
Subtotal 150,902 100 142,486 100 5,921 100 2,495 100

NOTE: (X) = suppressed to avoid disclosing information about particular individuals.

SOURCE: Social Security Administration, Master Beneficiary Record (communication with SSA, April 4, 2021).

Suggested Citation:"1 Introduction." National Academies of Sciences, Engineering, and Medicine. 2022. Selected Immune Disorders and Disability. Washington, DC: The National Academies Press. doi: 10.17226/26595.
×

TABLE 1-2 Supplemental Security Income (SSI) Beneficiaries Under Age 65, by Non-HIV Immune System Primary Diagnostic Group, December 2019

Diagnostic group
Inflammatory arthritis 26,411
Sjögren’s syndrome, polymyositis and dermatomyositis, systemic lupus erythematosus, systemic sclerosis, and undifferentiated and mixed connective tissue disease 14,344
Immune deficiency disorders, excluding HIV infection 1,995
Gout 1,724
Ankylosing spondylitis or other spondyloarthropathies 1,299
Systemic vasculitis 448
Total 46,221

SOURCE: Social Security Administration, Master Beneficiary Record (communication with SSA, April 4, 2021).

  1. of the dorsolumbar or cervical spine at 45 degrees or more) that is not surgically treatable, or age 46 1/2 or older.6

The committee notes that there are not specifically defined automatic diary date categorizations for other non-HIV immune conditions under consideration that do not improve, such as myositis with full muscle atrophy.

The committee describes the appeal process and diary date determination because the lifelong courses of the immune disorder diseases under consideration in this report combined with the diseases’ pattern of flares and remission can make it difficult for examiners to get a full understanding of the disease’s impact on a person’s ability to work. Flares can be precipitated by emotional or physical stress and include symptoms of swelling, joint pain, and fatigue. Flares can also be exacerbated by employment, particularly physically or emotionally demanding jobs or those that involve environmental exposures, such as cold and humid environments or direct sunlight. The timing of the disease’s initial onset, flares, and remission may affect the perceived severity of the disease and thus influence the determination or adjudication of the ability to earn at a level consistent with substantial gainful activity. As will be discussed in the next chapter, fatigability, or susceptibility to fatigue, is a common characteristic of the immune system disorders discussed in this report; as such, individuals may be unable to consistently engage in substantial gainful activity due to their fatigability whether or not they are experiencing acute fatigue at the time of disability determination.

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6https://secure.ssa.gov/apps10/poms.nsf/lnx/0426525045 (accessed January 12, 2022).

Suggested Citation:"1 Introduction." National Academies of Sciences, Engineering, and Medicine. 2022. Selected Immune Disorders and Disability. Washington, DC: The National Academies Press. doi: 10.17226/26595.
×

APPROACH TO THE TASK

The committee directed the project staff to search the literature for current research that would enable the members to address their task. The staff initially reviewed more than 1,382 titles and abstracts; those were narrowed to about 435 studies, which the committee members carefully reviewed for relevance to the committee’s task. The review began with a search of online databases for U.S. and international English-language literature from 1990 through 2020. The search covered PubMed, Scopus, and SSA and the National Academies Press websites. Several other searches were conducted for the same years focusing on further specific questions relating to the disorders listed in the Statement of Task. Committee members and project staff identified additional literature and information using traditional academic research methods and online searches throughout the course of the study.

At the committee’s first meeting, as the members reviewed and discussed their task, they agreed that there were many issues that they thought important to discuss, such as comorbidities, flares and remission, medication side effects, how treatments might indicate the severity of illness, and the impact of COVID-19 on immune system disorders. The committee decided to discuss those issues early in the report as they are crosscutting issues related to all of the diseases presented in the report (see Chapter 2).

The committee notes that it was not tasked with examining issues related to access to care. In its Statement of Task to the committee, SSA included text indicating that it recognizes that people may have difficulty accessing care or particular forms of treatment; however, some do so successfully, and the agency receives information about those treatments in the medical records. SSA makes individual decisions on each case based on all the evidence it receives, but cannot take access into consideration.

The committee discussed the issues related to diagnosis, disease course, and treatment in each chapter. The committee notes that professionally accepted diagnostic criteria do not exist for these diseases; instead, diagnosis is based on classification criteria and clinical judgment. Additionally, the diagnosis of autoimmune diseases may be complicated since there can be a variety of complex symptoms affecting multiple organ systems. Those symptoms and treatments are discussed in each chapter.

ORGANIZATION OF THE REPORT

The report is organized into seven chapters, beginning with this introductory chapter which provides background information for the report and includes a discussion of functional limitations and disability. The next chapter discusses cross-cutting issues that occur across the range of immune disorders (Chapter 2). Each subsequent chapter is devoted to a specific

Suggested Citation:"1 Introduction." National Academies of Sciences, Engineering, and Medicine. 2022. Selected Immune Disorders and Disability. Washington, DC: The National Academies Press. doi: 10.17226/26595.
×

immune disorder, as requested in the Statement of Task: systemic lupus erythematosus (Chapter 3), scleroderma (Chapter 4), myositis (Chapter 5), inflammatory arthritis (Chapter 6), and Sjögren’s Syndrome (Chapter 7).

Each disease-focused chapter addresses the current status of the diagnosis, treatment, and prognosis of the condition based on published evidence; where evidence is lacking, the committee members have used their professional judgment. Finally, each chapter also contains a discussion of pediatric immune disorders which highlights any differences between the adult-onset and childhood-onset disease. The precise organization of the chapters varies somewhat from one to the next as each chapter is organized so as to best present the information for that chapter’s disease focus.

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The U.S. Social Security Administration (SSA) administers the Social Security Disability Insurance program and the Supplemental Security Income program. As part of their process, immune system disorders are evaluated under Listing of Impairments 14.00 for adults and 114.00 for children. At the request of the SSA, the National Academies of Sciences, Engineering, and Medicine assembled a committee to review selected conditions related to the immune system. In particular, the SSA was interested in the current status of the diagnosis, treatment, and prognosis of immune system disorders including systemic lupus erythematosus (SLE), scleroderma, polymyositis, Sjogren's syndrome/disease, and inflammatory arthritis.

This report provides an overview of the current status of the diagnosis, treatment, and prognosis of these immune system disorders in the U.S. population and the relative levels of functional limitation typically associated with them, common treatments, and other considerations.

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