Selected Immune Disorders and Disability (2022)
Chapter: Front Matter
Selected
Immune Disorders
and Disability
Committee on Selected Immune Disorders and Disability
Board on Health Care Services
Health and Medicine Division
A Consensus Study Report of
THE NATIONAL ACADEMIES PRESS
Washington, DC
www.nap.edu
THE NATIONAL ACADEMIES PRESS 500 Fifth Street, NW Washington, DC 20001
This activity was supported by a contract between the National Academy of Sciences and the Social Security Administration (Contract No. 28321318D00060015). Any opinions, findings, conclusions, or recommendations expressed in this publication do not necessarily reflect the views of any organization or agency that provided support for the project.
International Standard Book Number-13: 978-0-309-68949-6
International Standard Book Number: 0-309-68949-X
Digital Object Identifier: https://doi.org/10.17226/26595
Library of Congress Control Number: 2022943145
Additional copies of this publication are available from the National Academies Press, 500 Fifth Street, NW, Keck 360, Washington, DC 20001; (800) 624-6242 or (202) 334-3313; http://www.nap.edu.
Copyright 2022 by the National Academy of Sciences. All rights reserved.
Printed in the United States of America
Suggested citation: National Academies of Sciences, Engineering, and Medicine. 2022. Selected immune disorders and disability. Washington, DC: The National Academies Press. https://doi.org/10.17226/26595.
The National Academy of Sciences was established in 1863 by an Act of Congress, signed by President Lincoln, as a private nongovernment institution to advise the nation on issues related to science and technology. Members are elected by their peers for outstanding contributions to research. Dr. Marcia McNutt is president.
The National Academy of Engineering was established in 1964 under the charter of the National Academy of Sciences to bring the practices of engineering to advising the nation. Members are elected by their peers for extraordinary contributions to engineering. Dr. John L. Anderson is president.
The National Academy of Medicine (formerly the Institute of Medicine) was established in 1970 under the charter of the National Academy of Sciences to advise the nation on medical and health issues. Members are elected by their peers for distinguished contributions to medicine and health. Dr. Victor J. Dzau is president.
The three Academies work together as the National Academies of Sciences, Engineering, and Medicine to provide independent, objective analysis and advice to the nation and conduct other activities to solve complex problems and inform public-policy decisions. The National Academies also encourage education and research, recognize outstanding contributions to knowledge, and increase public understanding in matters of science, engineering, and medicine.
Learn more about the National Academies of Sciences, Engineering, and Medicine at www.nationalacademies.org.
Consensus Study Reports published by the National Academies of Sciences, Engineering, and Medicine document the evidence-based consensus on studies’ statements of task by committees of experts. Reports typically include findings, conclusions, and recommendations based on information gathered by the committees and the committees’ deliberations. Each report has been subjected to a rigorous and independent peer-review process and represents the position of the National Academies on its statement of task.
Proceedings published by the National Academies of Sciences, Engineering, and Medicine chronicle the presentations and discussions at workshops, symposia, or other events convened by the National Academies. The statements and opinions contained in proceedings are those of the participants and are not endorsed by other participants, the planning committee, or the National Academies.
For information about other products and activities of the National Academies, please visit www.nationalacademies.org/about/whatwedo.
COMMITTEE ON SELECTED IMMUNE DISORDERS AND DISABILITY
JUDITH GREEN-McKENZIE (Chair), Professor of Medicine and Executive Director for Health, Safety and Environment, Johns Hopkins Health System and Johns Hopkins University
PHILIP ARMOUR, Senior Economist and Professor of Policy Analysis, Pardee RAND Graduate School
MATTHEW L. BASIAGA, Assistant Professor of Pediatrics, Mayo Clinic Alix School of Medicine
JOAN M. BATHON, Professor of Medicine and Chief, Division of Rheumatology, Columbia University Irving Medical Center/New York Presbyterian Hospital
LISA CHRISTOPHER-STINE, Associate Professor of Medicine and Neurology and Director of the Johns Hopkins Myositis Center, Johns Hopkins University School of Medicine
SHARON DOWELL, Associate Professor of Medicine, Division of Rheumatology, Howard University College of Medicine
AMANDA BETH FEINSTEIN, Clinical Assistant Professor, Department of Anesthesiology, Perioperative, and Pain Medicine, Stanford University School of Medicine
ANNETTE L. FITZPATRICK, Research Professor, Departments of Family Medicine, Epidemiology, and Global Health, University of Washington School of Medicine and Public Health
PATRICIA KATZ, Professor of Medicine and Health Policy, Division of Rheumatology, University of California San Francisco
KAREN BRANDT ONEL, Professor of Clinical Pediatrics and Chief, Division of Pediatric Rheumatology, Hospital for Special Surgery, Weill Cornell Medicine
AMI A. SHAH, Associate Professor of Medicine and Associate Director, Division of Rheumatology, Co-Director, Johns Hopkins Scleroderma Center, Johns Hopkins University School of Medicine
Consultant
NANETTE K. WENGER, Professor of Medicine Emeritus, Division of Cardiology, Emory University School of Medicine
Study Staff
CAROLYN FULCO, Scholar
BERNICE CHU, Program Officer
JOSEPH GOODMAN, Senior Program Assistant
BLAKE REICHMUTH, Associate Program Officer
SHARYL NASS, Senior Director, Board on Health Care Services
Reviewers
This Consensus Study Report was reviewed in draft form by individuals chosen for their diverse perspectives and technical expertise. The purpose of this independent review is to provide candid and critical comments that will assist the National Academies of Sciences, Engineering, and Medicine in making each published report as sound as possible and to ensure that it meets the institutional standards for quality, objectivity, evidence, and responsiveness to the study charge. The review comments and draft manuscript remain confidential to protect the integrity of the deliberative process.
We thank the following individuals for their review of this report:
Although the reviewers listed above provided many constructive comments and suggestions, they were not asked to endorse the conclusions or recommendations of this report nor did they see the final draft before its release. The review of this report was overseen by CYNTHIA D. MULROW, University of Texas Health Science Center, and B. NED
CALONGE, Colorado School of Public Health. They were responsible for making certain that an independent examination of this report was carried out in accordance with the standards of the National Academies and that all review comments were carefully considered. Responsibility for the final content rests entirely with the authoring committee and the National Academies.
3 SYSTEMIC LUPUS ERYTHEMATOSUS
Clinical Features, Diagnosis, and Disease Course
Disease-Specific Functional Limitations
Clinical Features, Diagnosis, and Disease Course, 94
Disease-Specific Functional Limitations
Clinical Features, Diagnosis, and Disease Course
Disease-Specific Functional Limitations
7 SJÖGREN’S DISEASE/SYNDROME
Clinical Features, Diagnosis, and Disease Course
Disease-Specific Functional Limitations
Acronyms and Abbreviations
| Ab | autoantibody |
| ACA | anti-centromere antibody |
| ACE | angiotensin-converting enzyme |
| ACLE | acute cutaneous lupus erythematosus |
| ACPA | anti-citrullinated protein antibody |
| ACR | American College of Rheumatology |
| AECG | American–European Consensus Group |
| ANA | anti-nuclear antibody |
| anti-MDA5 | anti-melanoma differentiation-association protein 5 |
| anti-Ro | anti-rhodopsin |
| AOSD | adult-onset Stills’ disease |
| APLA | antiphospholipid antibody |
| APLS | antiphospholipid syndrome |
| ARB | angiotensin II receptor blocker |
| ARS | anti-aminoacyl tRNA synthetase |
| ASTIS trial | Autologous Stem Cell Transplantation Internal Scleroderma |
| BAFF | B cell activating factor |
| bDMARD | biologic disease-modifying anti-rheumatic drug |
| BILAG-2004 | British Isles Lupus Assessment Group–2004 |
| CADM | clinically amyopathic dermatomyositis |
| CARRA | Childhood Arthritis and Rheumatology Research Alliance |
| CCLE | chronic cutaneous lupus erythematosus |
| CDC | Centers for Disease Control and Prevention |
| CDR | continuing disability review |
| CHAQ | Children’s Health Assessment Questionnaire |
| CK | creatine kinase |
| CMS | Centers for Medicare & Medicaid Services |
| CNS | central nervous system |
| CREST syndrome | calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia |
| CRP | C-reactive protein |
| cSLE | childhood-onset systemic lupus erythematosus |
| csDMARD | conventional synthetic disease-modifying anti-rheumatic drug |
| CT | computed tomography |
| CTD-PAH | connective tissue disorders–pulmonary arterial hypertension |
| DALY | disability-adjusted life-year |
| DAS28 | Disease Activity Score 28 |
| dcSSc | diffuse cutaneous systemic sclerosis |
| DHEA | dehydropiandrosterone |
| DLE | discoid lupus erythematosus |
| DM | dermatomyositis |
| DMARD | disease-modifying anti-rheumatic drug |
| dsDNA | double-stranded DNA |
| DU | digital ulcer |
| EDTA | European Dialysis and Transplant Association |
| EMG | electromyography |
| ERA | European Renal Association |
| ERA | enthesitis-related arthritis |
| ESR | erythrocyte sedimentation rate |
| ESRD | end stage renal disease |
| ESSDAI | EULAR Sjögren’s Syndrome Disease Activity Index |
| ESSPRI | EULAR Sjögrens Syndrome Patient Reported Index |
| EULAR | European Alliance of Associations for Rheumatology |
| EUSTAR | European Scleroderma Trials and Research |
| FDA | U.S. Food and Drug Administration |
| FI2 | functional index 2 test |
| FVC | forced vital capacity |
| GAD-7 | General Anxiety Disorder-7 |
| GAVE | gastric antral vascular ectasia |
| GC | glucocorticoid |
| G-CSF | granulocyte colony stimulating factor |
| GI | gastrointestinal |
| GRAPPA | Group for Research and Assessment in Psoriasis and Psoriatic Arthritis |
| GRCS | global rank composite score |
| HAQ | Health Assessment Questionnaire |
| HAQ-DI | Health Assessment Questionnaire–Disability Index |
| HCQ | hydroxychloroquine |
| HFrEF | reduced ejection fraction |
| HIV | human immunodeficiency virus |
| IBD | inflammatory bowel disease |
| IBM | inclusion-body myositis |
| ICF | International Classification of Functioning |
| IIM | idiopathic inflammatory myopathy |
| IL | interleukin |
| ILAR | International League of Associations for Rheumatology |
| ILD | interstitial lung disease |
| IMACS | International Myositis Assessment and Clinical Studies Group |
| IMNM | immune-mediated necrotizing myopathy |
| IV | intravenous |
| IVIG | intravenous immunoglobulin |
| JADI | Juvenile Arthritis Damage Index |
| JAK | janus kinase |
| JDM | juvenile dermatomyositis |
| JIA | juvenile idiopathic arthritis |
| JIIM | juvenile idiopathic inflammatory myopathy |
| JKI | janus kinase inhibitor |
| JPM | juvenile polymyositis |
| lcSSc | limited cutaneous systemic sclerosis |
| LN | lupus nephritis |
| LV | left ventricular |
| MAA | myositis-associated antibody |
| MAS | macrophage activation syndrome |
| MDA | melanoma differentiation-associated gene |
| MDA-5 | anti-melanoma differentiation associated gene-5 |
| MIPS | Merit-Based Incentive Payment System |
| MMF | mycophenolate mofetil |
| MMT | manual muscle test |
| MRI | magnetic resonance imaging |
| mRSS | modified Rodnan skin score |
| MSA | myositis-specific autoantibody |
| NAM | necrotizing autoimmune myopathy |
| NIH | National Institutes of Health |
| NPF | National Psoriasis Foundation |
| NSAID | nonsteroidal anti-inflammatory drug |
| NXP | nuclear matrix protein |
| NXP-2 | nuclear matrix protein-2 |
| OMERACT | Outcome Measures in Rheumatoid Arthritis Clinical Trials |
| PAH | pulmonary arterial hypertension |
| PDE-5 | phosphodiesterase-5 |
| PH | pulmonary hypertension |
| PhGA | Physician Global Assessment |
| PHQ-9 | Patient Health Questionnaire-9 |
| PM | polymyositis |
| PsA | psoriatic arthritis |
| PsARC | Psoriatic Arthritis Response Criteria |
| PsJIA | juvenile psoriatic arthritis |
| pSS | primary Sjögren’s syndrome |
| PT/OT | physical therapy and occupational therapy |
| RA | rheumatoid arthritis |
| RCT | randomized controlled trial |
| REVEAL | Registry to Evaluate Early and Long-term PAH Disease Management |
| RF | rheumatoid factor |
| RFC | residual functional capacity |
| RNP | ribonucleoprotein |
| RP-ILD | rapidly progressive interstitial lung disease |
| SAE | small ubiquitin-like modifier-activating enzyme |
| SCLE | subacute cutaneous lupus erythematosus |
| SHAQ | Scleroderma Health Assessment Questionnaire |
| SHAQ-DI | Scleroderma Health Assessment Questionnaire–Disability Index |
| SLAM-R | Revised Systemic Lupus Activity Measure |
| SLE | systemic lupus erythematosus |
| SLEDAI-2K | Systemic Lupus Erythematosus Disease Activity Index-2K |
| SLICC | System Lupus Erythematosus International Collaborating Clinics Group |
| SLS I | Scleroderma Lung Study I |
| SLS II | Scleroderma Lung Study II |
| SMS | Systemic Manifestation Score |
| SPIN | Scleroderma Patient-Centered Intervention |
| SRC | scleroderma renal crisis |
| SS | Sjögren’s syndrome |
| SSA | Social Security Administration |
| SSc | systemic sclerosis |
| SSDI | Social Security Disability Insurance |
| SSI | Supplemental Security Income |
| SSRI | selective serotonin reuptake inhibitor |
| sSS | secondary Sjögren’s syndrome |
| SWSF | stimulated whole salivary flow rate |
| T2T | treat-to-target |
| TIF | transcription intermediary factor |
| TNF | tumor necrosis factor |
| UWSF | unstimulated whole salivary flow rate |
| VLA | valued life activity |
| WHO | World Health Organization |
