Systemic scleroderma
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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