Systemic Sclerosis (SSc)

Systemic sclerosis (SSc) is a rare autoimmune disorder of the connective tissue characterized by widespread fibrosis and vascular abnormalities that cause systemic damage to the skin and multiple organ systems. There are no cures or medications that can prevent progressive deterioration. Surgery is considered when organ involvement is severe and other treatments have failed.¹

Surgical management for patients with systemic sclerosis (SSc) includes interventions such as esophageal procedures, digital sympathectomy, lung transplantation, and amputation, which are best avoided and used only as a last resort. This is due to SSc-associated complications, such as poor tissue perfusion, and the high risk of wound healing problems and infections. Thus, surgical options are indicated only when nonsurgical measures are unsuccessful and the risk-to-benefit ratio of these procedures has been carefully evaluated. In addition, specific perioperative measures should be considered to reduce the surgical risk.¹

Surgical Considerations

Because SSc is an autoimmune condition, medical therapy includes systemic corticosteroids, which increase the probability of postoperative wound infections. Thus, the chances of slow wound healing must be factored into the decision for surgical intervention. In addition, reduced tissue perfusion may increase the chances of intraoperative and postoperative wound complications.¹ 

The risk-to-benefit ratio of surgery should be carefully evaluated for every patient. Efforts should be taken to improve the chances of success and minimize complications. Due to vasodilatory effects and superior pain control, local or regional anesthesia is preferred over general anesthesia, which can be difficult to administer due to SSc-related facial involvement. Health care providers should pay special attention to patients’ needs, such as maintaining a warm temperature during surgery (to prevent cold-induced vasospasm) and providing adequate intravenous hydration.¹

Read more about SSc clinical features 

Facial Surgery

Bilateral Commissurotomy

Patients with severe microstomia may require bilateral commissurotomy, which is performed in rare cases to increase the width of the mouth.²

Gastrointestinal Surgery

Surgical procedures such as antrectomy and venting gastrectomy are used only when patients with SSc have extreme gastrointestinal complications, such as intractable vomiting or anorexia, due to the major complications that can occur with these techniques.^3-5

Esophageal Surgery

Esophagectomy should only be performed in select patients with long-segment fibrotic strictures who are not responsive to endoscopic dilation therapy. This is because this procedure poses a high risk of significant postoperative complications, anastomotic leak or stricture, and mortality.³

Roux-en-Y gastric bypass has been shown to be a safe and effective surgical option to alleviate bloating, diarrhea, dysphagia, and acid reflux in patients with SSc. However, long-term follow-up studies are needed to evaluate postoperative reflux, dysphagia, remission, and pulmonary function in these patients.³

Minimally invasive laparoscopic fundoplication has been found to improve gastroesophageal reflux disease (GERD) with acceptable dysphagia postoperatively.³

Surgery for Gastric Antral Vascular Ectasia

Autologous hematopoietic stem cell transplantation (HSCT) with prior high-dose chemotherapy has shown benefit in SSc patients with gastric antral vascular ectasia (GAVE). Like other surgical interventions, HSCT is performed only in patients who are resistant to less invasive therapies such as endoscopic therapies, sclerotherapy, cryotherapy, and intravenous Cytoxan® (cyclophosphamide).^3-5

Surgery for Anorectal Dysfunction

In case of severe rectal prolapse, surgical resection of the rectum and sigmoid colon is performed.⁴

Read more about SSc complications

Hand Surgery

Hand surgery may be performed to correct severe flexion contractures or sclerodactyly. For digital vasculopathy, surgical interventions include digital sympathectomy, debulking of areas of calcinosis, and deep surgical debridement.^6-8

Digital Sympathectomy

Digital sympathectomy involves mechanical denervation to decrease vasospastic sympathetic tone as well as adventitial removal of periarterial fibrosis. In refractory cases that are potentially at risk of digit loss due to ischemia, digital (or peripheral) sympathectomy may provide benefit by decreasing digital pain and ulceration and eliminating the need for amputation with minor postoperative complications.^6-8

Surgical Excision of Calcinosis

Surgical excision of calcinosis by an experienced surgeon is indicated if these lesions are recurrent, severely painful, infected, or causing hand dysfunction. It involves removal of localized or diffuse calcinotic lesions. While localized deposits can be completely excised, resulting in resolution of the ulcer and wound healing, diffuse lesions cannot be completely excised, so surgery is aimed at debulking the main calcium deposits.⁸

Read more about SSc comorbidities

Kidney Transplantation

Kidney transplantation is an option for SSc patients with end-stage renal failure. However, the overall survival and graft survival post-transplantation are poorer in these patients than in those with other primary kidney diseases.⁹

Read more about SSc treatment

Lung Transplantation

Lung transplantation is performed in select patients with SSc who are unresponsive to medical therapy, do not have extrapulmonary contraindications, and have pulmonary arterial hypertension (PAH), severe interstitial lung disease (ILD), or rapid progressive SSc. A systematic review evaluating the survival of patients with SSc after lung transplantation demonstrated that short- and intermediate-term survival rates post-transplantation were similar to those of patients with PAH and ILD without SSc.^10,11

Read more about SSc therapies

References

1. Carr ZJ, Klick J, McDowell BJ, Charchaflieh JG, Karamchandani K. An update on systemic sclerosis and its perioperative management. Curr Anesthesiol Rep. 2020;10(4):512-521. doi:10.1007/s40140-020-00411-8
2. Albilia JB, Lam DK, Blanas N, Clokie CML, Sándor GKB. Small mouths … big problems? A review of scleroderma and its oral health implications. J Can Dent Assoc. 2007;73(9):831-836.
3. Aiolfi A, Nosotti M, Matsushima K, et al. Surgical treatment of recalcitrant gastroesophageal reflux disease in patients with systemic sclerosis: a systematic review. Langenbecks Arch Surg. 2021;406(5):1353-1361. doi:10.1007/s00423-021-02118-8
4. Emmanuel A. Current management of the gastrointestinal complications of systemic sclerosis. Nat Rev Gastroenterol Hepatol. 2016;13(8):461-472. doi:10.1038/nrgastro.2016.99
5. Gyger G, Baron M. Systemic sclerosis: gastrointestinal disease and its management. Rheum Dis Clin North Am. 2015;41(3):459-473. doi:10.1016/j.rdc.2015.04.007
6. Satteson ES, Chung MP, Chung LS, Chang J. Microvascular hand surgery for digital ischemia in scleroderma. J Scleroderma Relat Disord. 2020;5(2):130-136. doi:10.1177/2397198319863565
7. Momeni A, Sorice SC, Valenzuela A, Fiorentino DF, Chung L, Chang J. Surgical treatment of systemic sclerosis–is it justified to offer peripheral sympathectomy earlier in the disease process? Microsurgery. 2015;35(6):441-446. doi:10.1002/micr.22379
8. Beldner S, Rabinovich RV, Polatsch DB. Scleroderma of the hand: evaluation and treatment. J Am Acad Orthop Surg. 2020;28(16):e686-e695. doi:10.5435/JAAOS-D-19-00547
9. Bertrand D, Dehay J, Ott J, et al. Kidney transplantation in patients with systemic sclerosis: a nationwide multicentre study. Transpl Int. 2017;30(3):256-265. doi:10.1111/tri.12923
10. Khan IY, Singer LG, de Perrot M, et al. Survival after lung transplantation in systemic sclerosis. A systematic review. Respir Med. 2013;107(12):2081-2087. doi:10.1016/j.rmed.2013.09.015
11. Cottin V, Brown KK. Interstitial lung disease associated with systemic sclerosis (SSc-ILD). Respir Res. 2019;20(1):13. doi:10.1186/s12931-019-0980-7

Reviewed by Hasan Avcu, MD, on 5/3/2023.

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Erum Naqvi, PhD

Erum Naqvi obtained her Ph.D. in Molecular Medicine from Hannover Medical School (Germany) after completing her Masters in Biomedical Science and Bachelors in Microbiology from University of Delhi (India). She has several years of experience as a science writer.