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Suggested Citation:"Summary." 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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Summary

The U.S. Social Security Administration (SSA) administers the Social Security Disability Insurance program and the Supplemental Security Income program. Those programs provide disability benefits to individuals who qualify due to a disability. To qualify for disability benefits, an individual must meet SSA’s statutory definition of disability. SSA categorizes impairments into 14 body systems for adults and 15 body systems for children in an appendix to the governing regulations called the Listing of Impairments. Immune system disorders are evaluated under Listing of Impairments 14.00 for adults and 114.00 for children. SSA requested that the National Academies of Sciences, Engineering, and Medicine assemble a committee to review selected conditions related to the immune system. In particular, SSA was 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:

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):

Suggested Citation:"Summary." National Academies of Sciences, Engineering, and Medicine. 2022. Selected Immune Disorders and Disability. Washington, DC: The National Academies Press. doi: 10.17226/26595.
×
  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.
  2. 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).
  3. 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.
  4. For the immune system disorders identified in task 1, provide a summary of select treatments currently being studied in clinical trials.
  5. 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 function
Suggested Citation:"Summary." National Academies of Sciences, Engineering, and Medicine. 2022. Selected Immune Disorders and Disability. Washington, DC: The National Academies Press. doi: 10.17226/26595.
×
  1. ing 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.

The report will include conclusions but not recommendations.

In the report, when terms such as “severity” or “severe” are used, the committee shall identify to the degree possible whether the term is being used with SSA’s program definition or as it is used in the clinical or research settings, so as to avoid reader confusion, and, if necessary, will specify the pertinent differences between the two definitions.

The committee shall not describe issues with respect to access to treatments due to financial circumstances, including insurance limitations. While SSA recognizes some patients may have difficulty accessing care or particular forms of treatment due to financial circumstances, others do successfully access those treatments. SSA may receive information about those treatments in the medical records SSA considers when making disability determinations and conducting continuing disability reviews. SSA understands improvement is not certain in all cases. SSA makes individual decisions on each case based on all the evidence they receive.

OVERALL FINDINGS AND CONCLUSIONS

Immune disorders contribute substantially to morbidity, mortality, and health care costs in the United States. They are the highest 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 immune disorders, which are frequently chronic. 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.1 Immune disorders may be diagnosed in childhood or in adulthood. 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. Three overall characteristics are notable among the immune disorders that the committee studied: their systemic and heterogeneous nature, their cyclic and unpredictable disease course, and the effects of their treatment.

First, immune disorders are systemic diseases with heterogeneous symptoms that involve multiple organ systems and commonly also involve

___________________

1 M. D. Rosenblum, I. K. Gratz, J. S. Paw, and A. K. Abbas. 2012. Treating human autoimmunity: Current practice and future prospects. Science Translational Medicine 4(125)125sr1.

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

general symptoms. All of the disorders are associated with a number of potentially disabling complications across multiple body systems, such as the pulmonary, cardiac, gastrointestinal, endocrine, and vascular systems. Some are also associated with a higher risk of cancer. Complications involving other body systems can be an indicator of disease severity. Additionally, general symptoms such as depression and anxiety, pain, fatigue, and cognitive effects are common in individuals with immune disorders and can have a profound impact on an individual’s functioning as well as adherence to treatment. Functional impairments2 can co-occur and interact with each other or the underlying immune disorder. For example, fatigue, weakness (i.e., psychomotor slowing), sleep dysfunction, and low appetite may contribute to depression or to the effects of inflammation from the underlying immune disorder, all of which may contribute to functional impairments. The varied disease symptoms, effects on multiple body systems, and wide range of functional limitations can make diagnosis of immune disorders difficult. Diagnosis may also be complicated when patients have co-occurring immune disorders, called overlap syndromes. The diseases most commonly involved in overlap syndromes include rheumatoid arthritis, lupus, scleroderma, and myositis.

Second, the cyclic recurrence and unpredictability of these diseases is important to understand. Many immune disorders are characterized by fluctuations in disease activity—periods of quiescence interrupted by periods of greater disease activity, or flares. Each episode or flare can lead to increased disease progression, functional impairment and permanent organ or joint damage. Further long-term complications can accrue due to toxicities from the medications (such as glucocorticoids) that are used to treat the flare. Patients may experience fluctuations in disease that are impactful yet are not persistent or severe enough to meet clinical or research definitions. The “bad days” are an indication of fluctuating disease activity and can be associated with increased disease progression, functional impairment, depression, diminished quality of life, or organ damage. Some precipitating factors for flares have been identified, though they are still mostly unknown, which further contributes to the unpredictable nature of immune disorders.

Finally, immune disorders cannot be cured. Instead, the goal of treatment is to have the lowest degree of disease activity to prevent or limit organ damage. Functional impairment is usually due to active inflammation or organ damage and can result from multiple systemic effects

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2 While it is customary in medical terminology to refer to “functional impairment,” SSA distinguishes between impairments (which are the diagnosable medical conditions) and functional limitations (which are associated with those impairments). In this report, “functional impairment” refers to the medical definition of the term meaning limitations associated with a primary diagnosis (e.g., psychological, cognitive, or physical impairments) that hinder a person’s ability to perform everyday actions without assistance.

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

or comorbidities occurring simultaneously. Glucocorticoids are used as a first-line therapy in many cases because of their global action on multiple inflammatory pathways and because of their rapid onset of action; they can be used concurrently with disease-modifying anti-rheumatic drugs (DMARDs). Many of the drugs commonly used to treat immune disorders are considered off-label drugs. That does not mean that the drugs are “experimental”; rather, off-label drugs are U.S. Food and Drug Administration (FDA)-approved, but not approved to treat a specific immune disorder. Providers constantly assess the benefit-to-risk ratio of DMARD therapies with the primary goal of treating the underlying condition and minimizing the risk of side effects. Careful monitoring of glucocorticoid therapy, combination therapy, and the addition of drugs to treat multiple systemic effects may be indicators of a more severe disease. Additionally, the use of intravenous glucocorticoids and cyclophosphamide may indicate acute and severe decompensation of a newly diagnosed or underlying immune disorder. The responsiveness to different medications varies greatly among patients, and it can be challenging to find medications with the greatest efficacy. Although inflammation may improve, often there is irreparable damage, and functioning may not improve. Most patients require long-term treatment (i.e., greater than 12 months), likely for a lifetime.

DISEASE-SPECIFIC FINDINGS AND CONCLUSIONS

Systemic Lupus Erythematosus (SLE)

SLE is a chronic, potentially fatal autoimmune disease with several phenotypes which are characterized by inflammation of different tissues of the body, including the skin, joints, kidneys, lungs, and nervous system. The clinical features may vary from mild to severe and life-threatening. People with SLE often have functional impairments that lead to difficulties in physical functioning. The impact of SLE on physical functioning depends largely on the severity of the disease activity and on which disease manifestations are present, with severe fatigue, neurocognitive symptoms, and musculoskeletal symptoms often associated with the greatest difficulties. There can be times of active disease, called flares, and times where the disease is mostly quiescent, called remission. About 20 percent of people with SLE develop the disease before 20 years of age. It is rare to get SLE before age 5. SLE is more common in females than in males and in certain ethnic groups, including African American, Hispanic, South and Southeast Asian, and American Indian/Alaska Native populations, than in white populations.

Diagnosing SLE can be challenging because symptoms are nonspecific and overlap with other common diseases, and no single clinical feature or

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

laboratory test can confirm the diagnosis. Diagnosis is based on numerous signs and symptoms and laboratory tests. Histopathology, imaging, and specific autoantibodies also aid in diagnosis. Antinuclear antibodies (ANAs) are the hallmark of the disease, and a test for them using an immunofluorescence assay should be the initial test performed when SLE is suspected. Although a positive ANA test is seen in more than 97 percent of cases of SLE, the specificity of the test is poor (only 20 percent), and positive results may also be seen in patients with other disorders and in healthy populations.

The disease course in SLE is variable, both between patients and within the same patient, and often unpredictable. However, there are three distinct patterns of disease activity: intermittent flares (relapsing–remitting), chronically active disease, and quiescent disease. A relapsing–remitting course is most common. More than half of patients with SLE will have a disease flare after achieving a state of low disease activity or quiescence. Most flares are mild to moderate in severity and may involve one or more organ systems, most frequently the mucocutaneous, musculoskeletal, renal, hematologic, or immunologic systems. Almost one-third, however, are severe, with significant associated morbidity. Most severe flares follow a moderate flare in the same organ system, and about one in five severe flares will manifest with new organ involvement or worsening of major organ disease.

Treatment depends on the organ systems involved, and despite advancements in SLE treatment and a better understanding of the disease, patients have significant morbidity and mortality. Survival rates are 85 to 90 percent during the first 10 years; the leading causes of mortality include cardiovascular disease, infections, and renal disease. Traditional therapies have included hydroxychloroquine, systemic glucocorticosteroids and conventional immunosuppressive drugs, and biologic agents (e.g., belimumab). Additionally, there is ongoing research testing novel therapies that target, for example, interferons, cytokines and their receptors, intracellular signals, plasma cells, and T lymphocytes.

A diagnosis of SLE in childhood is an important risk factor for morbidity and mortality. The increased risk is attributed to organ damage and adverse effects from medications, particularly long-term steroid use. Childhood SLE has negative effects on growth and development and interferes with social and cognitive skills.

Scleroderma

Systemic sclerosis (SSc), also called scleroderma, is a complex, multisystem rheumatic condition that is highly variable in disease presentation and trajectory. It is characterized by immune dysregulation, vasculopathy, and progressive fibrosis that typically affects the skin and internal organs.

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

Internal organ involvement can be minimal, ranging from mild gastroesophageal reflux, subtle skin changes, and Raynaud’s phenomenon to severe and fulminant multisystem disease with interstitial lung disease, severe digital ischemia, severe gastrointestinal (GI) dysmotility, myopathy, renal crisis, and pulmonary hypertension. Patients with systemic sclerosis may develop functional impairment due to skin thickening, small and large joint contractures, inflammatory arthritis, tendon friction rubs, myositis, digital ulcers, GI dysmotility, and dyspnea from cardiopulmonary complications. Skin thickening, joint contractures, synovitis, and digital ulcers in particular may contribute to hand disability. Systemic sclerosis has the highest case-specific mortality and major morbidity of any of the autoimmune rheumatic diseases. The mortality and morbidity of SSc is typically the result of complications such as cardiopulmonary, renal, GI, and peripheral vascular involvement, and interstitial lung disease and pulmonary hypertension are the most common causes of death. Although SSc can affect children and adults at any age, the peak onset is between 40 and 50 years old, and the disease occurs more frequently in women. Scleroderma may also be more severe in African Americans and Native Americans.

Early diagnosis and close surveillance for internal organ involvement may improve patient outcomes, and treatment strategies are aimed at improving quality of life and halting the progression of complications. Diagnosis can be made through a careful history, physical examination, and testing for scleroderma-specific autoantibodies. It is important to appreciate that scleroderma is a very heterogeneous disease with both clinical and laboratory prognosticators available to define the expected disease course. Defining the extent of skin involvement (none, limited, or diffuse) and testing for autoantibodies are useful clinical tools that may aid in diagnosis, disease classification, and risk stratification. Cutaneous subsets and autoantibody type can provide important information about a patient’s potential disease course and prognosis. Improved clinical phenotyping and early evaluation for active occult organ disease are important for identifying appropriate treatment options.

Frequent monitoring through physical examination, laboratory testing, and serial pulmonary function testing and echocardiogram assessments can identify patients with complications at an early stage, offering an opportunity for intervention that may improve the prognosis. There are no current conventional treatments that are effective in stopping or reversing the overall course of systemic sclerosis. Several medications have been demonstrated to slow the progression of symptoms or to reduce the development of new symptoms. In addition to medications that are used to treat individual symptoms or complications, some medications may interrupt the disease process in a variety of ways. Immunosuppressive drugs, used alone or in combination, may be used to control disease activity and

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

slow the progression of the disease, particularly for patients with active cutaneous, pulmonary, and musculoskeletal manifestations. Vasoactive medications and drugs targeting GI manifestations are also commonly used. Although there are no treatments that will cure scleroderma, there are many treatment options to improve both quality of life and survival rates.

Myositis

Myositis is a collection of rare diseases characterized by inflammation and weakness of the skeletal muscles, and it can also involve internal organs such as the lungs, heart, and esophagus. This report discusses three types of myositis that are common in clinical practice: dermatomyositis (DM), polymyositis (PM), and juvenile dermatomyositis (JDM). DM primarily affects the skin, muscles, joints, and lungs, whereas PM mostly affects the muscles, usually with no skin involvement. DM is also strongly associated with cancers. JDM shares many clinical features with adult DM, though in JDM, interstitial lung disease (ILD) and cancer are rarer, while GI, endocrine, and vascular effects are more common. Functional disability is common in individuals with myositis, particularly impaired muscle function, predominantly in the proximal and axial muscle groups, that results in functional limitations related to muscle weakness and decreased endurance with more fatigability.

A diagnosis of myositis is based on a combination of a patient’s history, clinical examination, and laboratory tests such as creatine kinase and muscle biopsy. Additional testing may include myopathic features on electromyography and the detection of myositis-specific autoantibodies (MSAs). The diagnostic workup is similar in adults and children, but children frequently do not undergo a muscle biopsy when the characteristic rashes of JDM are present. In recent years, MSAs have been increasingly used as additional evidence to make a diagnosis, select treatments, and predict disease course by, for example, providing information about disease severity, the risk of systemic effects, and cancer. More research and standardization still need to be done in this field.

The usual course of myositis is progressive, but it can be relapsing and remitting with periods of worsening and quiescence. The disease course can be categorized as: monophasic, meaning a disease in which the patient can be off medication over time; chronic, meaning the patient continues to require medication; or polyphasic, meaning the patient experiences relapse and remission throughout the disease course. In rare cases, even after long periods of quiescence (i.e., years), the disease may still flare. The disease course and outcomes vary, and a prognosis depends on systemic effects and multimorbidity. In cases where muscle inflammation leads to irreversible

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

muscle atrophy and fatty replacement, permanent muscle weakness in those targeted muscle groups will persist due to permanent damage. ILD is a well-recognized complication of DM and PM and worsens the prognosis of a patient with the disease. Cardiac involvement is also associated with poor prognosis. Other potential longstanding complications include joint deformities and contractures, which may occur more often in children. Even if the disease is controlled, patients are often left with damage such as calcinosis, muscle atrophy with fatty replacement leading to permanent weakness, and decreased endurance.

Corticosteroids with adjunctive steroid-sparing immunosuppressive therapies are recommended to treat disease activity, prevent mortality, and reduce long-term disability. While they are effective, corticosteroids lead to numerous complications, including hypertension, cataracts, osteoporosis, weight gain, and psychosis. In JDM, chronic glucocorticoid exposure can have an impact on a child’s growth, leading to short stature and pubertal delay as well as having a significant psychosocial impact. Combinations of second-line therapies or newer third-line therapies are used in severe, refractory, or corticosteroid-dependent diseases. Treatments should be chosen according to the patient’s clinical features and systemic involvement. Future trials may consider subgrouping patients into clinical and serological subtypes to help identify biomarkers for response to specific immunosuppressive and biological agents. Prompt diagnosis and treatment generally lead to better outcomes, decreased permanent damage, and decreased long-term disability. However, in many patients, morbidity can be significant, and a permanent reduction in muscle mass and skin damage can continue to affect quality of life.

Inflammatory Arthritis

Inflammatory arthritis is a broad term for a group of chronic autoimmune diseases characterized by pain, swelling, warmth, and tenderness in joints and stiffness that lasts for an hour or more. Most inflammatory forms of arthritis include systemic effects such as skin rashes, eye inflammation, hair loss, and dry mouth. Unlike the more common osteoarthritis, or arthritis that is caused by physical wear and tear of a joint over time, inflammatory forms of arthritis present in children as well as adults. In 2019, inflammatory arthritis accounted for 60 percent of all Social Security disability beneficiaries receiving benefits for immune system disorders, not including HIV. This report discusses three types of inflammatory arthritis: rheumatoid arthritis (RA), psoriatic arthritis (PsA), and juvenile idiopathic arthritis (JIA).

RA is a chronic autoimmune inflammatory disease that predominantly affects the joints. However, given its systemic nature, it may also cause

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

fever, weight loss, marked fatigue, and muscle atrophy as well as other organ-specific features such as scleritis, accelerated cardiovascular disease, and ILD. The arthritis of RA, as with any inflammatory arthritis, typically presents with joint pain and swelling, and prolonged stiffness in the early morning. Early RA may present in a single joint (monoarthritis) or a few joints (oligoarthritis) but characteristically tends to evolve into a symmetrical polyarthritis of small and large joints which, if left untreated or inadequately treated, will cause damage to articular structures and disability. The musculoskeletal impairments associated with RA are more commonly the most disabling and the major source of functional limitations for individuals with this condition. In addition, RA-associated ILD may cause severe respiratory impairment leading to the need for a lung transplant or even death. RA may also influence global functioning through other mechanisms such as fatigue.

RA is a chronic disease with daily discomfort, and patients also frequently have disease flares. During disease flares, patients experience worsening inflammation, which results in an exacerbation of joint pain and swelling and the potential for new joint damage. In patients with longstanding and severe disease, persistent synovial inflammation will result in the erosion of cartilage and bone, leading to joint destruction and deformities, which in turn cause chronic pain and functional limitations. The goal of RA treatment is to attain and maintain control of disease activity in an effort to reduce the symptoms of joint pain and swelling, prevent deformity, maintain quality of life and function, and limit extra-articular disease manifestations. Pharmacologic treatments, specifically DMARDs, are the mainstay of therapy as they limit progressive joint damage and improve function.

PsA is a chronic inflammatory disease of the joints, spine, and entheses. It may affect other tissues as well (e.g., dactylitis, nail involvement, sacroiliitis, enthesitis) and most commonly occurs in association with psoriasis, an autoimmune skin disease manifested by erythematous plaques covered by silvery scales. Skin manifestations commonly precede the arthritis. PsA has heterogeneous clinical manifestations, so gauging its effects and improvement from treatment must be done through several types of measures. The principal measures used to assess response to treatment and remission for PsA are largely the same as those used for RA and represent composite, multidimensional outcome measures incorporating clinical data, functional assessment, patient-reported symptoms, and global assessment.

JIA, formerly known as juvenile rheumatoid arthritis, is an umbrella term for several types of inflammatory arthritis that begin in childhood. The subtypes of JIA share the common features of joint pain and inflammation with the adult inflammatory arthropathies. By convention, JIA pertains to children less than 16 years of age with joint inflammation present for

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

6 continuous weeks. JIA is the most common chronic rheumatic disease of childhood, and it affects approximately 300,000 children in the United States. Once an individual has been diagnosed with JIA, the diagnosis remains for the full extent of disease throughout the individual’s lifespan.

Sjögren’s Syndrome

Sjögren’s syndrome (SS), also known as Sjögren’s disease, is a systemic autoimmune disease characterized by glandular dysfunction resulting in sicca symptoms (dryness, usually in the eyes and mouth), fatigue and chronic musculoskeletal pain, and possible systemic disease with a significant risk of progression to non-Hodgkin’s lymphoma. It is the second most common autoimmune disorder after RA. SS is associated with significant functional impairment caused by reduced visual function, pain, fatigue, anxiety, and depression.

A diagnosis requires evidence of glandular dysfunction and serologic or pathologic evidence that SS is the cause of the glandular dysfunction. SS may be difficult to recognize, and, because of that, diagnosis can be delayed by more than 10 years. The disease course of SS is slowly progressive and unlikely to improve, with the exception of extra-glandular involvement. Patients will accrue advancing gland damage, leading to varying levels of decreased exocrine gland function, and can also accumulate additional autoimmune diagnoses. As with other immune disorders, disease activity can fluctuate over time and progress between flares and remissions, particularly in the case of salivary gland inflammation. Once gland disease develops, it is generally accepted that disease remission is not attainable, and symptoms of dryness may be static.

There are no treatments to cure SS, and treatment research lags behind that of other autoimmune disorders. Management involves improving quality of life by reducing dryness, fatigue, and chronic pain, and by treating systemic manifestations to prevent damage accrual, which will worsen the vital and functional prognosis. The treatment of manifestations of sicca, fatigue, and pain mainly involves symptomatic measures and rehabilitation, and generally immunosuppressants are not recommended to treat those symptoms. Therapy must be tailored to the specific organs involved and to the severity of the disorder. Mild manifestations can be treated with hydroxychloroquine or local corticosteroids, while moderate to severe systemic involvement will require the use of systemic corticosteroid therapy, often combined with steroid-sparing immune modulation.

Juvenile SS is a rare and poorly defined disease that persists into adulthood, with a mean age at diagnosis of 10 years. As with the adult disease, the hallmarks of juvenile SS involve exocrine gland inflammation with eventual loss of function along with the potential for extra-glandular disease. It

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

is not known if juvenile SS is an early presentation of adult-type disease or if it is a different entity, as is the case for other autoimmune diseases. Differences in presenting symptoms between children and adults suggest it is the latter. A variety of organ systems may be affected, which may result in numerous manifestations, including neurological, dermatological, musculoskeletal, vascular, gastrointestinal, respiratory, renal, and hematological. A unique aspect of childhood SS is a lower prevalence of sicca and higher incidence of recurrent or persistent parotitis.

Suggested Citation:"Summary." 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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Suggested Citation:"Summary." 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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Suggested Citation:"Summary." 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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Suggested Citation:"Summary." 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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Suggested Citation:"Summary." 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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Suggested Citation:"Summary." 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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Suggested Citation:"Summary." 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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Suggested Citation:"Summary." 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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Suggested Citation:"Summary." 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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Suggested Citation:"Summary." 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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Suggested Citation:"Summary." 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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Suggested Citation:"Summary." 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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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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