Systemic scleroderma

Rare. Connective tissue pathology which affects mainly women between 40 and 60 years. It combines a dysfunction of the immune cells (autoimmune disease), fibroblasts (increased production of proteins of the extracellular matrix) and endothelial cells.

Clinical forms:

-        diffuse cutaneous form (40 % of cases): the skin damage extends beyond the elbows and knees. evolution is usually quick with a significant visceral involvement (pulmonary, renal, digestive involvement); There are no anticentromeres antibodies

-        limited cutaneous form (60 %): the skin damage does not go beyond the elbows and knees; anticentromeres antibodies are present in 70-90 % of cases; visceral manifestations are rare, but there is a risk of pulmonary hypertension

-        scleroderma is sometimes associated with other connective tissue diseases: systemic lupus erythematosus, Sjogren syndrome, inflammatory arthritis, inflammatory myopathy; the term 'overlap syndrome' is used in those cases.

Clinical picture:

-        Raynaud's phenomenon (3 phases: syncopal, asphyxial and erythromelalgic)

-        sclerosis of the skin and the mucous membranes: thin fingers in slight flexion, xerostomia, gradual decrease in mouth opening

-        diffuse interstitial lung disease:  inflammatory injury following with destruction and interstitial fibrosis: crackles, dyspnea, restrictive syndrome, decreased DLCO

-        pulmonary arterial hypertension (10-15 %): due to vasoconstriction (defect of NO production), local thrombosis and pulmonary vascular wall remodeling

Treatment: symptomatic; corticosteroids and immunosuppressants in severe cases


Anesthetic implications:

1) preoperative: echocardiography; oximetry at room air, X-rays or recent CTscan of the chest; pulmonary function (+ DLCO); renal function; corticosteroids ?

2) intraoperative:

-        difficult peripheral venous access 

-        full stomach/esophagus situation

-        intubation: small mouth opening

-        prudent artificial ventilation

-        prophylactic broad spectrum antibiotics

-        avoid all potential causes of increase in pulmonary vascular resistance; management of pulmonary arterial hypertension

-        avoid hypothermia to avoid triggering a Raynaud's syndrome

3)  postoperative: major risk for respiratory complications

4)  locoregional anesthesia: a few instances of prolonged duration of the sensitive block have been reported


References : 

-        Tung A, Sweitzer B, Cutter T.
Cardiac arrest after labetalol and metoclopramide administration in a patient with scleroderma.
Anesth Analg 2002 ; 95 : 1667-8

-        Bégneu E, Aïssou M, Lescot T, Cabane J-P, Beaussier M. 
Prise en charge périopératoire du patient sclérodermique. 
Ann Fr Anesth Réanim 2014 ; 33 : 669-76.

-        Lee GY, Cho S.
Spinal anesthesia for cesarean section in a patient with systemic sclerosis associated with interstitial lung disease.
Korean J Anesthesiol 2016; 69: 406-8.


Updated: September 2019