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Care Team

Systemic sclerosis (SSc) is a rare, chronic autoimmune disease of connective tissue characterized by widespread fibrosis affecting the skin, vasculature, and multiple internal organs.¹ Given the multisystemic nature of SSc, patients require multidisciplinary care for optimal management of their condition. For the treatment of each affected organ or organ system, coordinated care by multiple specialists is required.²

Dermatological Care

Skin involvement is one of the primary clinical manifestations of SSc. Symptoms may include sclerodactyly, calcinosis cutis, digital ulceration, and Raynaud phenomenon. Some of these clinical manifestations may become severe enough for gangrene to develop or amputation to be required.² Microstomia is another complication affecting the skin, in which fibrosis of the perioral skin causes the opening of the mouth to become progressively smaller. Compromised nutrition is a consequence of this problem.^2,3

Read more about SSc clinical features

Members of the multidisciplinary team who manage the dermatological manifestations of SSc include the following specialists²:

Dermatologists
Rheumatologists
Hand therapists
Maxillofacial surgeons
Specialized nurses

Cardiovascular Care

Involvement of the cardiovascular system is another, potentially more severe clinical feature of SSc. Myocardial fibrosis and inflammatory damage, pulmonary arterial hypertension, cardiac arrhythmias and pericardial effusion are many of the possible signs and symptoms of SSc-mediated cardiovascular problems.²

Read more about SSc complications

Members of the multidisciplinary team who manage the cardiovascular manifestations of SSc include the following specialists²:

Cardiologists
Rheumatologists
Pulmonologists

Renal Care

Renal crisis and end-stage renal failure are life-threatening complications of SSc. SSc-related renal crisis presents as severe hypertension rapidly leading to acute renal failure, congestive heart failure, hypertensive encephalopathy, and/or microangiopathic hemolytic anemia.⁴ Renal crisis that is not controlled by treatment can progress to end-stage renal failure, which requires dialysis or renal transplant.⁵

Read more about SSc treatment

Members of the multidisciplinary team who manage the renal manifestations of SSc include the following specialists^4,5:

Nephrologists
Rheumatologists
Transplant surgeons
Specialized nurses

Respiratory Care

The lungs are often affected in SSc as a consequence of fibrosis and the development of interstitial lung disease and pulmonary arterial hypertension. Patients with SSc may be considered for a lung transplant depending on the severity and rate of decline in their pulmonary function.²

Read more about SSc surgical management

Members of the multidisciplinary team who manage the pulmonary manifestations of SSc include the following specialists²:

Pulmonologists
Rheumatologists
Radiologists
Cardiologists
Respiratory therapists
Transplant surgeons

Gastrointestinal Care

Gastrointestinal symptoms are often the early manifestations of SSc disease, including gastroesophageal reflux, esophageal dysmotility, gastric antral vascular ectasia, abdominal distension secondary to small-intestinal bacterial overgrowth (SIBO), and malnutrition due to microstomia.²

Members of the multidisciplinary team who manage the gastrointestinal manifestations of SSc include the following specialists²:

Gastroenterologists
Rheumatologists
Maxillofacial surgeons
Nutritionists or dietary specialists

Read more about SSc diet and nutrition

Musculoskeletal Care

Joint involvement is common in patients with SSc and variably manifests as arthralgia, arthritis, joint erosions, restricted mobility due to fibrosis of periarticular structures, tendonitis, myalgia, muscle weakness, and the formation of calcifications within the joints.²Some treatments may decrease inflammation and improve the strength of muscles and tissues surrounding involved joints; however, the restricted joint mobility and contractures that develop as a consequence of progressive SSc fibrosis cannot be treated successfully with physical therapy and other interventions.²

Members of the multidisciplinary team who manage the musculoskeletal manifestations of SSc include the following specialists²:

Rheumatologists
Orthopedists/orthopedic surgeons
Physical therapists
Hand therapists

Reproductive System Care

SSc may affect the reproductive organs of both men and women. Men with SSc may experience erectile dysfunction,⁶ whereas women with SSc may experience dyspareunia, vaginal dryness, and frequent bacterial or fungal infections secondary to intercourse. Pregnancy may also present unique challenges in women with SSc.²

Members of the multidisciplinary team who manage the reproductive manifestations of SSc include the following specialists²:

Rheumatologists
Urologists
Obstetricians/gynecologists

Other SSc Care Providers

SSc may increase the risk of cancer, infection, and psychological disorders. Providers who may be called on to manage these complications or aspects of SSc include the following specialists²:

Oncologists
Infectious disease specialists
Psychologists/counselors

References

Adigun R, Goyal A, Hariz A. Systemic sclerosis. StatPearls [Internet]. Updated May 8, 2022. Accessed April 26, 2023.
Farina N, Benanti G, De Luca G, et al. The role of the multidisciplinary health care team in the management of patients with systemic sclerosis. J Multidiscip Healthc. 2022;15:815-824. doi:10.2147/JMDH.S295478
Türk I, Cüzdan N, Çiftçi V, Arslan D, Ünal I. Correlations between clinical features and mouth opening in patients with systemic sclerosis. Arch Rheumatol. 2020;35(2):196-204. doi:10.46497/ArchRheumatol.2020.7434
Vaidya PN, Basyal B, Finnigan NA. Scleroderma and renal crisis. StatPearls [Internet]. Updated January 22, 2023. Accessed April 26, 2023.
Lavergne A, Pladys A, Couchoud C, Lassalle M, Vigneau C. Systemic sclerosis and end-stage renal disease: study of patient characteristics, follow-up and outcomes in France. J Nephrol. 2021;34(2):617-625. doi:10.1007/s40620-020-00746-9
Foocharoen C, Tyndall A, Hachulla E, et al. Erectile dysfunction is frequent in systemic sclerosis and associated with severe disease: a study of the EULAR Scleroderma Trial and Research group. Arthritis Res Ther. 2012;14(1):R37. doi:10.1186/ar3748

Reviewed by Harshi Dhingra, MD, on 4/30/2023.

Maria Arini Lopez, PT, DPT, CSCS, CMTPT, CIMT

Maria Arini Lopez, PT, DPT, CSCS, CMTPT, CIMT is a freelance medical writer and Doctor of Physical Therapy from Maryland. She has expertise in the therapeutic areas of orthopedics, neurology, chronic pain, gastrointestinal dysfunctions, and rare diseases especially Ehlers Danlos Syndrome.

Systemic Sclerosis (SSc)