Systemic sclerosis. Part II: perioperative considerations.
2023; Elsevier BV; Volume: 23; Issue: 3 Linguagem: Inglês
10.1016/j.bjae.2022.10.003
ISSN2058-5357
AutoresC.I. Efrimescu, Suzanne Donnelly, Donal J. Buggy,
Tópico(s)Inflammatory Myopathies and Dermatomyositis
ResumoLearning objectivesBy reading this article you should be able to:•Detail the features of systemic sclerosis of relevance to anaesthesia.•Formulate an appropriate plan for assessment and investigations before surgery.•Discuss the advantages and risks of different anaesthetic techniques in systemic sclerosis patients.•Anticipate and manage the potential challenges during surgery.•Predict the possible difficulties for postoperative management and their potential impact on outcomes.Key points•Systemic sclerosis can affect all aspects of perioperative care, including vascular access, airway management, intraoperative medications and choice of anaesthesia.•Approximately 75% of patients have impaired lung function, gastrointestinal and skin involvement within the first 2 yrs of disease onset.•Detecting and mitigating subclinical severe cardiac and respiratory comorbidity is paramount during preoperative assessment.•Invasive ventilation for respiratory failure in patients with interstitial lung disease and systemic sclerosis is associated with a high risk of death.•Scleroderma renal crisis and catastrophic antiphospholipid syndrome are rare potential life-threatening postoperative complications. By reading this article you should be able to:•Detail the features of systemic sclerosis of relevance to anaesthesia.•Formulate an appropriate plan for assessment and investigations before surgery.•Discuss the advantages and risks of different anaesthetic techniques in systemic sclerosis patients.•Anticipate and manage the potential challenges during surgery.•Predict the possible difficulties for postoperative management and their potential impact on outcomes. •Systemic sclerosis can affect all aspects of perioperative care, including vascular access, airway management, intraoperative medications and choice of anaesthesia.•Approximately 75% of patients have impaired lung function, gastrointestinal and skin involvement within the first 2 yrs of disease onset.•Detecting and mitigating subclinical severe cardiac and respiratory comorbidity is paramount during preoperative assessment.•Invasive ventilation for respiratory failure in patients with interstitial lung disease and systemic sclerosis is associated with a high risk of death.•Scleroderma renal crisis and catastrophic antiphospholipid syndrome are rare potential life-threatening postoperative complications. Systemic sclerosis (SSc) is an uncommon disease that involves multiple organ systems. Advances in our understanding of the pathophysiology and progress in organ-specific management strategies have decreased mortality. The 5- and 10-yr survival rates has been recently estimated at 86% and 72%, respectively.1Pokeerbux M.R. Giovannelli J. Dauchet L. et al.Survival and prognosis factors in systemic sclerosis: data of a French multicenter cohort, systematic review, and meta-analysis of the literature.Arthritis Res Ther. 2019; 21: 86Crossref PubMed Scopus (100) Google Scholar This improved long-term survival increases the likelihood of encountering patients who are older and present a high burden of non-lethal associated features and complications. The multisystem nature of the disease affects most aspects of perioperative care. Hence, a thorough understanding of preoperative assessment and optimisation and of the role of the anaesthetist are critical for improved postoperative outcomes. The epidemiology, pathophysiology, diagnosis and current disease management have been described in a recent accompanying paper in this journal.2Efrimescu C. Donnelly S. Buggy D.J. Systemic sclerosis. Part I: epidemiology, diagnosis and therapy.BJA Educ. 2023; 23Abstract Full Text Full Text PDF Scopus (1) Google Scholar This article focuses on the perioperative management and disease-specific postoperative complications. Because SSc is a relatively infrequent condition among patients presenting for surgery, there is little, if any, high-level research evidence on optimal perioperative management, so current practice based on expert opinion and consensus. Most patients will have an established diagnosis of SSc when presenting for surgery. Preoperative assessment should consider the urgency and complexity of surgery; the history, extent and severity of disease; the opinions of a rheumatologist; specific drug therapies; and symptoms suggestive of cardiac and pulmonary involvement. The yearly frequency of specialist review (i.e. >twice/year) is an indicator of disease severity and the involvement of internal organs.3Hachulla E. Agard C. Allanore Y. et al.French recommendations for the management of systemic sclerosis.Orphanet J Rare Dis. 2021; 16: 322Crossref PubMed Scopus (22) Google Scholar Collecting all this information relies on a multidisciplinary approach, bringing together the surgeon, rheumatologist, cardiologist and pulmonary medicine physician under the coordination of the anaesthesia team. An ‘organ system’ approach should be followed, and careful consideration should be given to the potential anaesthetic issues detailed in Supplementary Table S1. This approach may uncover signs and symptoms of unrecognised subclinical involvement, which carries significant morbidity and could be unmasked by the perioperative stress response. History and examination should be oriented towards assessing the disease extent and severity. Symptoms and signs for relevant manifestations should be sought. These include dysphagia and reflux oesophagitis (almost universally present); other disorders that may increase the risk of aspiration (e.g. diabetes, achalasia); limited range of movement or contractures involving relevant joints (e.g. neck, mouth opening, limbs); and cardiac, respiratory and renal involvement. Point-of-care assessment can be challenging as simple measurements such as pulse oximetry and non-invasive arterial BP measurement may be technically difficult and unreliable in the presence of Raynaud's phenomenon (RP) and scleroderma. Internal organs are involved much earlier than was previously appreciated. Approximately 75% of patients have impaired lung function (diffusing capacity for carbon monoxide [DLCO] <80%), and involvement of the gastrointestinal (GI) system and skin within 2 yrs of disease onset.4Jaeger V.K. Wirz E.G. Allanore Y. et al.Incidences and risk factors of organ manifestations in the early course of systemic sclerosis: a longitudinal EUSTAR study.PLoS One. 2016; 11e0163894Crossref Scopus (140) Google Scholar Supplementary Table S1 describes manifestations of SSc by organ system. Skin changes (thickening, loss of normal folds, telangiectasia, pigmentation and depigmentation) may develop before, or in parallel with visceral involvement.5Yazdany J. Manno R. Hellmann D.B. et al.Scleroderma (systemic sclerosis).in: Papadakis M.A. McPhee S.J. Rabow M.W. Current medical diagnosis & treatment 2021. McGraw-Hill Education, New York, NY2021Google Scholar The extent of skin involvement (above/below knees and elbows) should be noted during the preoperative assessment as being indicative of the limited compared with the diffuse cutaneous subtype. Raynaud's phenomenon is a consistent finding in these patients and can precede other findings by years.2Efrimescu C. Donnelly S. Buggy D.J. Systemic sclerosis. Part I: epidemiology, diagnosis and therapy.BJA Educ. 2023; 23Abstract Full Text Full Text PDF Scopus (1) Google Scholar Extensive or rapidly progressive skin disease is a marker of severity and is usually associated with the diffuse cutaneous systemic sclerosis (dcSSc) phenotype, cardiac and lung involvement. Features such as ulceration, subcutaneous calcinosis and superinfection contribute to morbidity by causing chronic pain and loss of movement and function in the extremities, especially the hand. Ischaemia and necrosis can result in autoamputation of digital tufts, and in extreme cases, phalanges. Even limited skin involvement may be locally severe (e.g. thick adherent skin and digital tuft resorption). Scleroderma may recede and soften as the disease progresses and in later-stage disease, may become fragile requiring gentle skin manoeuvring. Cardiac manifestations are a consequence of extracellular matrix deposition leading to fibrosis and microcirculatory failure, decreased cardiac reserve and decreased left ventricular mass.6Rosato E. Gigante A. Gasperini M.L. et al.Nutritional status measured by BMI is impaired and correlates with left ventricular mass in patients with systemic sclerosis.Nutrition. 2014; 30: 204-209Crossref PubMed Scopus (18) Google Scholar Subclinical cardiac disease is common, and a low threshold should be kept for stress testing and advanced/invasive investigations. The absence of respiratory symptoms should not be considered reassuring. Interstitial lung disease (ILD) is an early complication and may be detectable years before the onset of symptoms. The recent change in the mean pulmonary artery pressure (mPAP) at rest used to define pulmonary arterial hypertension (PAH) (mPAP ≥20 mmHg) affects the interpretation of previous data in SSc, which used the older definition (mPAP at rest ≥25 mmHg). Because it is a heterogenous disease, SSc with PAH may belong to any of the classifications groups but is most frequently classified as connective tissue disease (CTD) PAH. The time from initial diagnosis of PAH should be considered during preoperative assessment in addition to current treatment as an indicator for disease progression and severity. Although limited cutaneous SSc (lcSSc) was traditionally associated with PAH, more recent studies suggest a similar cumulative incidence in dcSSc.7Nihtyanova S.I. Schreiber B.E. Ong V.H. et al.Prediction of pulmonary complications and long-term survival in systemic sclerosis.Arthritis Rheumatol. 2014; 66: 1625-1635Crossref PubMed Scopus (328) Google Scholar In addition, up to one-third of patients may present sleep-disordered breathing with nocturnal baseline mean arterial oxygen SpO2 1 g day−1 is uncommon and suggests primary glomerular disease. The standard preoperative investigations should include full blood count, renal and coagulation profile and ECG as a baseline. In contrast to other rheumatic diseases, these patients may not have increased inflammatory markers (e.g. erythrocyte sedimentation rate [ESR], C-reactive protein [CRP]) during active disease phases. Persistent increases in ESR and CRP correlate with a decline in lung function and PAH.10Ross L. Stevens W. Rabusa C. et al.The role of inflammatory markers in assessment of disease activity in systemic sclerosis.Clin Exp Rheumatol. 2018; 36: 126-134PubMed Google Scholar Gadolinium-enhanced cardiac MRI is increasingly useful for diagnosis of subclinical cardiac SSc (e.g. myocardial fibrosis), which may otherwise reveal itself in the postoperative period.11Denton C.P. Khanna D. Systemic sclerosis.Lancet. 2017; 390: 1685-1699Abstract Full Text Full Text PDF PubMed Scopus (1147) Google Scholar All patients should undergo annual screening for PAH: pulmonary function tests (PFTs) (i.e. DLCO) and transthoracic echocardiography (TTE).12Coghlan J.G. Denton C.P. Grunig E. et al.Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study.Ann Rheum Dis. 2014; 73: 1340-1349Crossref PubMed Scopus (558) Google Scholar A DLCO <60% predicted value is suggestive of PAH regardless of ILD status.3Hachulla E. Agard C. Allanore Y. et al.French recommendations for the management of systemic sclerosis.Orphanet J Rare Dis. 2021; 16: 322Crossref PubMed Scopus (22) Google Scholar The DETECT algorithm (Fig. 1) is useful in prioritising those who require echocardiography, particularly where resources are limited.12Coghlan J.G. Denton C.P. Grunig E. et al.Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study.Ann Rheum Dis. 2014; 73: 1340-1349Crossref PubMed Scopus (558) Google Scholar Echocardiography may miss up to 30% of PAH patients in this subgroup.13Hinchcliff M. Khanna S. Hsu V.M. et al.Survival in systemic sclerosis-pulmonary arterial hypertension by serum autoantibody status in the pulmonary hypertension assessment and recognition of outcomes in scleroderma (PHAROS) registry.Semin Arthritis Rheum. 2015; 45: 309-314Crossref PubMed Scopus (17) Google Scholar Where findings on TTE can suggest PAH, confirmatory right heart catheterisation is required. A repeat preoperative TTE is indicated in those with SSc PAH, specific cardiac risk factors or known cardiac involvement with no recent stratification. There should be a low threshold for performing PFTs where relevant, as abnormal function may be present with minimal respiratory symptoms and a normal chest X-ray. The presence of airway disease on PFTs is useful for preoperative stratification in those patients with minimal SSc symptoms.14Carr Z.J. Klick J. McDowell B.J. et al.An update on systemic sclerosis and its perioperative management.Curr Anesthesiol Rep. 2020; 10: 512-521Crossref PubMed Scopus (6) Google Scholar High-resolution CT and DLCO measurements should be undertaken in all patients suspected of having ILD. The following are predictive for ILD: anti-Scl-70/anti-topoisomerase 1 antibody, DLCO <70% predicted, reduced forced vital capacity (FVC) with a normal or increased forced expiratory volume in the first second/forced vital capacity (FEV1/FVC) ratio, increased ESR and CRP, and decreased performance on the 6-min walk test. ILD extent can be classified as limited (≤20% of the pulmonary surface on CT or FVC ≥70% when the CT is indeterminate) or extensive (≥20% of the pulmonary surface on CT or FVC ≤70% when the CT is indeterminate).15Goh N.S. Desai S.R. Veeraraghavan S. et al.Interstitial lung disease in systemic sclerosis: a simple staging system.Am J Respir Crit Care Med. 2008; 177: 1248-1254Crossref PubMed Scopus (821) Google Scholar An FVC <50% of predicted value suggests difficult mechanical ventilation weaning.3Hachulla E. Agard C. Allanore Y. et al.French recommendations for the management of systemic sclerosis.Orphanet J Rare Dis. 2021; 16: 322Crossref PubMed Scopus (22) Google Scholar The autoantibody profile may predict some disease features and potential complications. Anti-centromere antibodies are more frequently associated with lcSSc and PAH. Anti-Scl-70/anti-topoisomerase 1 is characteristically found in dcSSc and is associated with ILD and cardiac involvement. Anti-RNA polymerase III autoantibody is associated with higher risk of SRC and silent anaemia secondary to gastric antral vascular ectasia, whereas anti-U3-RNP is associated with a higher risk of cardiac involvement and PAH.4Jaeger V.K. Wirz E.G. Allanore Y. et al.Incidences and risk factors of organ manifestations in the early course of systemic sclerosis: a longitudinal EUSTAR study.PLoS One. 2016; 11e0163894Crossref Scopus (140) Google Scholar,5Yazdany J. Manno R. Hellmann D.B. et al.Scleroderma (systemic sclerosis).in: Papadakis M.A. McPhee S.J. Rabow M.W. Current medical diagnosis & treatment 2021. McGraw-Hill Education, New York, NY2021Google Scholar,11Denton C.P. Khanna D. Systemic sclerosis.Lancet. 2017; 390: 1685-1699Abstract Full Text Full Text PDF PubMed Scopus (1147) Google Scholar A summary of relevant investigations and their findings is presented in Table 1.Table 1Preoperative investigations. APTT, activated partial thromboplastin time; CPET, cardiopulmonary exercise testing; CRP, C-reactive protein; DLCO, diffusing capacity for carbon monoxide; ESR, erythrocyte sedimentation rate; FBC, full blood count; FEV1, forced expiratory volume in the first second; FVC, forced vital capacity; GI, gastrointestinal; ILD, interstitial lung disease; MPAP, mean pulmonary artery pressure; PAH, pulmonary arterial hypertension; PFTs, pulmonary function tests; PPIs, proton pump inhibitors; SRC, scleroderma renal crisis; TTE, transthoracic echocardiographyInvestigationsPossible findings/commentsFBC and coagulation•Anaemia of chronic disease and iron deficiency (chronic GI losses).•Microangiopathic haemolytic anaemia in SRC.•Prolonged APTT may suggest associated antiphospholipid antibodies/lupus anticoagulant.Biochemistry•Hypomagnesemia because of long-term PPIs.•Liver disfunction is seldomly present (may suggests associated primary biliary cirrhosis and sclerosing cholangitis).•Slightest abnormality of urea and creatinine should be investigated urgently as it can be an early predictor of SRC.•Increased creatine kinase should prompt consideration of an overlap syndrome.ESR/CRP•Usually not increased.•May suggest intercurrent infection or an inflammatory phase of disease (requires specialist follow-up).Autoantibodies•Not essential for preoperative assessment.•May predict some associated organ involvement or postoperative risks (see text).PFTs•ILD predictors: DLCO <70% predicted value.•PAH predictors: DLCO < 60% predicted value with preservation of volumes, reduced FVC with a normal or increased FEV1/FVC ratio.•Mechanical ventilation weaning difficulty predictors: FVC 36) and reduced survival (peak V˙ O2 < 15.6 ml kg−1 min−1 and V˙ E/V˙ CO2 slope > 35).Right heart catheterisation•PAH diagnosis cut off: mPAP ≥ 20 mmHg at rest.•Gold standard for PAH confirmation and clinical group identification.High-resolution lung CT•Consider in those with a DLCO suggestive of early ILD ( 5 mg day−1 for >4 weeks). Thickened, bound skin and flexion contractures, could make oscillometric arterial BP measurements difficult and unreliable. Invasive arterial pressure monitoring may be required even for uncomplicated surgery. Skin thickness, ulcerations and RP may decrease the peripheral pulse oximetry signal rendering it unreliable. Similarly, vasospasm/RP may affect beat-to-beat cardiac output/BP measurement derived from digital cuff and ‘volume clamp’ technology which relies on spectroscopy. In addition, digital cuff devices should be avoided as they may trigger vasospasm and lead to finger necrosis. Acute changes in the pulse oximetry signal quality and values can herald the development of RP (i.e. from poor temperature control, pain, perioperative stress or intraoperative medication). To decrease the risk of digital ulcers in patients with severe RP, the pulse oximetry probe should be moved regularly between different location.22Roberts J.G. Sabar R. Gianoli J.A. et al.Progressive systemic sclerosis: clinical manifestations and anesthetic considerations.J Clin Anesth. 2002; 14: 474-477Crossref PubMed Scopus (32) Google Scholar Peripheral venous access may require ultrasound guidance for cannulation. Central venous vascular access may be required for even uncomplicated surgeries when peripheral access cannot be secured reliably. Invasive arterial BP monitoring should preferentially use a large artery (e.g. brachial artery) to avoid RP and secondary ischaemia and necrosis. The use of transoesophageal echocardiography (TOE) may be warranted for intraoperative cardiac performance monitoring when cardiac involvement is suspected. Its use should be considered on a case-by-case basis after reviewing most recent gastroscopy results, as significant oesophageal involvement could increase the risk of oesophageal injury associated with probe insertion and manipulation. The presence of skin fibrosis (prone to pressure induced ulcers in late stages), decreased subcutaneous fat and associated contractures requires meticulous surgical positioning. In addition, corneal lubrication and careful eyes taping should be used as keratoconjunctivitis sicca and eyelid skin changes are common. Intraoperative and perioperative vasoconstrictive episodes can be prevented by mitigating inadvertent perioperative hypothermia, by maintaining the operating theatre temperature at >23°C, warm fluids infusion and convective air blankets. Although inadvertent perioperative hypothermia is common, a theoretical risk of intraoperative hyperthermia exists because of vasospasm and impaired sweating. Challenges include difficult mask ventilation (e.g. facial anatomical deformities), limited mouth opening and neck movement and aspiration risk. Microstomia, reduced temporomandibular joint movement, or both are responsible for reduced mouth opening. Neck movement restriction is a consequence of limited soft tissue flexibility from fibrosis rather than of cervical spine involvement. Hence, cervical spine plain radiologic imaging has limited value in airway management planning. Microstomia and a high Mallampati grade are ominous signs of a potentially difficult airway. Preoperative mouth physiotherapy during the month before surgery may add up to 1 cm to mouth opening, which may help airway management.3Hachulla E. Agard C. Allanore Y. et al.French recommendations for the management of systemic sclerosis.Orph
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