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
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International Standard Book Number-13: 978-0-309-68949-6
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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.
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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
Clinical Features, Diagnosis, and Disease Course
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 |
