Summary
Definition
History and exam
Key diagnostic factors
- presença de fatores de risco
- Fenômeno de Raynaud
- nódulos ou úlceras digitais
- edema das mãos e dos pés
- espessamento da pele
- perda de função das mãos
- esclerodactilia
- pirose, refluxo e disfagia
- distensão abdominal
- incontinência fecal
- artralgias e mialgias
- capilaroscopia anormal da dobra ungueal
- telangiectasia
- calcinose subcutânea
- dispneia
- estertores secos nas bases pulmonares
- crepitação tendínea
- hipertensão moderada/acentuada de início abrupto
Other diagnostic factors
- fadiga
- tosse seca
- tolerância reduzida ao exercício
- perda de peso
- artrite inflamatória
- fraqueza muscular proximal (miosite inflamatória)
- sinovite
- maior acentuação do componente pulmonar do som cardíaco B2
- sinais de anemia
Risk factors
- história familiar de esclerodermia
- desregulação imune (por exemplo, fator antinuclear [FAN] positivo)
- exposição a substâncias ambientais e toxinas (por exemplo, poeira de sílica ou solventes)
Diagnostic investigations
1st investigations to order
- autoanticorpos séricos
- Hemograma completo
- ureia e creatinina sérica
- Velocidade de hemossedimentação (VHS)
- proteína C-reativa
- microscopia da urina
- testes de função pulmonar completos (espirometria, volumes pulmonares e medição da capacidade de difusão)
- eletrocardiograma (ECG)
- ecocardiograma
- TC de alta resolução do tórax
- esofagografia baritada
Investigations to consider
- radiografia torácica
- endoscopia digestiva alta ± biópsia
- enzimas musculares séricas
- estudos de eletromiografia/condução nervosa
- biópsia muscular
Treatment algorithm
crise renal esclerodérmica
tamponamento cardíaco
Fenômeno de Raynaud: sem úlceras
Fenômeno de Raynaud: com desenvolvimento de úlcera digital
envolvimento generalizado da pele
Envolvimento gastrointestinal
miopatia
sinovite, crepitação tendínea ou artrite inflamatória
doença pulmonar intersticial
derrame pericárdico
hipertensão arterial pulmonar
Contributors
Authors
Maureen D. Mayes, MD, MPH
Professor of Medicine
Division of Rheumatology and Clinical Immunogenetics
University of Texas Health Science Center
Houston Medical School
Houston
TX
Disclosures
MDM is an advisory board member for Boehringer-Ingelheim, Galapagos, Corbus, the US Medical and Advisory of Scleroderma Foundation, and Eicos. MDM is on the grant review board for Medtelligence. She receives royalties from Oxford University Press and Henry Stewart Talks, and has been paid for consultancy to the Eicos Advisory Board. MDM is an author of a number of references cited in this topic.
Acknowledgements
Dr Maureen D. Mayes would like to gratefully acknowledge Dr Rajpreet K. Arora-Singh, a previous contributor to this topic. RKA declares that she has no competing interests.
Peer reviewers
Mary Ellen Csuka, MD
Associate Professor of Medicine
Department of Rheumatology
Medical College of Wisconsin
Milwaukee
WI
Disclosures
MEC has been reimbursed by: Actelion International (and is a member of its speakers bureau); Encysive Pharmaceuticals; and MediQuest Therapeutics. She is local principal investigator for the SCOT trial, sponsored by NIH.
Jerry Molitor, MD, PhD
Associate Professor
Division of Rheumatic and Autoimmune Diseases
Department of Medicine
University of Minnesota
Minneapolis
MN
Disclosures
JM declares that he has no competing interests.
Bridget Griffiths, MD, FRCP(UK)
Consultant Rheumatologist
Department of Rheumatology
Freeman Hospital
Newcastle Upon Tyne
UK
Disclosures
BG declares that she has no competing interests.
Ariane L. Herrick, MD, FRCP
Reader in Rheumatology and Consultant Rheumatologist
Musculoskeletal Research Group
University of Manchester
Manchester
UK
Disclosures
ALH declares that she has no competing interests.
Peer reviewer acknowledgements
BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.
Disclosures
Peer reviewer affiliations and disclosures pertain to the time of the review.
References
Key articles
van den Hoogen F, Khanna D, Fransen J, et al. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League Against Rheumatism collaborative initiative. Ann Rheum Dis. 2013 Nov;72(11):1747-55.Full text Abstract
Kowal-Bielecka O, Fransen J, Avouac J, et al. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis. 2017 Aug;76(8):1327-39.Full text Abstract
Baron M, Bernier P, Côté LF, et al. Screening and therapy for malnutrition and related gastro-intestinal disorders in systemic sclerosis: recommendations of a North American expert panel. Clin Exp Rheumatol. 2010 Mar-Apr;28(2 Suppl 58):S42-6.Full text Abstract
American College of Rheumatology. 2023 American College of Rheumatology (ACR) guideline for the treatment of interstitial lung disease in people with systemic autoimmune rheumatic disease. Aug 2023 [internet publication].Full text
Iudici M, van der Goes MC, Valentini G, et al. Glucocorticoids in systemic sclerosis: weighing the benefits and risks - a systematic review. Clin Exp Rheumatol. 2013 Mar;31(2 Suppl 76):157-65. Abstract
Johnson SR, Brode SK, Mielniczuk LM, et al. Dual therapy in IPAH and SSc-PAH. A qualitative systematic review. Respir Med. 2012 May;106(5):730-9. Abstract
Reference articles
A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
Differentials
- Fenômeno de Raynaud primário
- Esclerodermia localizada (morfeia)
- Doença mista do tecido conjuntivo
More DifferentialsDiretrizes
- Guideline for the treatment of interstitial lung disease in people with systemic autoimmune rheumatic disease
- Treatment of systemic sclerosis - associated interstitial lung disease
More Diretrizes
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