Oxford Handbook of Anaesthesia (3 edn)
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Rheumatoid arthritis
Rheumatoid arthritis (RA) is a chronic, systemic inflammatory disorder mainly involving joints but with extra-articular effects. Overall prevalence in the UK is 1–2% but it is 3 times higher in females. Peak onset is between 30 and 55yr. It also affects children as Still's disease. Patients are often frail, in chronic pain, and taking medications with adverse effects. Airway problems are common. There is a higher than average mortality due to both the disease itself and the presence of concurrent disorders.
Preoperative assessment
Articular
Temporomandibular: assess for limited mouth opening.
Cricoarytenoid: fixation of the cricoarytenoid joints may lead to voice changes, hoarseness, or even rarely to stridor from glottic stenosis. Larynx can also be obstructed by amyloid or rheumatoid nodules. Minimal oedema may lead to airway obstruction postoperatively.
Atlantoaxial subluxation (AAS) occurs in ∼25% of severe rheumatoid patients, but of these only a quarter will have neurological signs or symptoms. Enquire about tingling hands or feet, neck pain, and assess range of neck movement. Excessive movement during anaesthesia may lead to cervical cord compression.
Anterior AAS: comprises 80% of all AAS. C1 forward on C2 from destruction of transverse ligament. Significant if there is a gap of >3mm between the odontoid and the arch of the atlas in lateral flexion radiographs. Worsened by neck flexion.
Posterior AAS: this is rare. C1 backward on C2 resulting from destruction of the odontoid peg. Can be seen on lateral extension radiographs. Worsened by neck extension (e.g. from direct laryngoscopy).
Vertical AAS: arises from destruction of lateral masses of C1. The odontoid moves upwards through foramen magnum to compress cervicomedullary junction.
Lateral AAS: uncommon. Arises from involvement of the C1/C2 facet joints. More than 2mm difference in lateral alignment is significant. Causes spinal nerve compression and vertebral artery compression. Requires a frontal open mouth odontoid view to assess.
Subaxial subluxation (i.e. below C2):
More than 2mm loss of alignment is significant.
Look for this particularly if patient has undergone previous fusion at a higher level.
Other joints: assess joint deformities with a view to positioning and possible anaesthetic technique (if planning an axillary block, can the patient abduct their arm?). Manual dexterity may be important if planning to use standard PCA apparatus after surgery. Special adaptations are available, e.g. trigger by blowing.
Non-articular
Cardiovascular: association with coronary artery disease. Systemic vasculitis may lead to arterial occlusion in various organs and Raynaud's. Myocardial disease due to fibrosis, amyloid or nodular involvement. Pericarditis and pericardial effusions uncommon. Aortic incompetence and endocarditis rare.
Respiratory: fibrosing alveolitis (restrictive defect), frequently asymptomatic. Acute pneumonitis rare. Pleural effusions and/or nodules in pleura may show on X-ray. Association with obliterative bronchiolitis. Costo-chondral disease gives reduced chest wall compliance.
Anaemia: NSAID-associated blood loss. Normocytic, normochromic anaemia of chronic disease. Drug-associated bone marrow depression. Felty's syndrome is a combination of splenomegaly and neutropenia and may be associated with anaemia and thrombocytopenia.
Nervous system: peripheral and compression neuropathies occur. Cord compression may be atlanto-axial or subaxial. Neurological changes may be chronic or acute (trauma).
Infections: common both from the disease itself and drug effects.
Renal and hepatic: chronic renal failure from drugs is common, as is decreased albumin, increased fibrinogen and A1-acid glycoprotein (acute phase protein).
Investigations
All patients should have FBC, U&Es, ECG, and CXR. Consider LFT, ABGs, and coagulation studies.
Cervical spine radiographs: the role of preoperative cervical spine flexion/extension views is controversial and interpretation is difficult. The automatic reordering of radiographs in all patients is unnecessary. Flexion/extension views are mandatory in all patients with neurological symptoms or signs, and in those with persistent neck pain. Stabilisation surgery may be necessary before other elective surgery is undertaken. Preoperative cervical spine radiographs may help determine management but only in association with a full clinical review. Specialist radiological advice should be sought. Unless it is certain that the cervical spine is stable, all rheumatoid patients should be treated as if they might have an unstable spine. This may involve awake fibreoptic intubation or manual in-line stabilisation when undertaking direct laryngoscopy/LMA insertion/moving the patient. MRI and CT may be useful in assessing cord compression.
Pulmonary function tests should be carried out for patients with unexplained dyspnoea or radiological abnormalities.
An ENT opinion should be sought and nasendoscopy performed if there is hoarseness or symptoms/signs of respiratory obstruction.
Echocardiography is needed if there is valvular or pericardial involvement and in symptomatic cardiac disease.
Drugs in the perioperative period
Steroid supplementation if indicated ( p. 172).
NSAIDs: continue as this enables early mobilisation. Stop if postoperative bleeding is a potential problem, hypotension, or deterioration in renal function.
Disease-modifying antirheumatoid drugs (DMARDs): these drugs include gold, penicillamine, and immunosuppressant drugs such as methotrexate and azathioprine. Usually continue, as mobilisation is important and there is little evidence that omission reduces postoperative complications (wound infections). If leucopenic consult with rheumatologist.
TNF A-blockers: etanercept, adalimumab, anakinra, and infliximab belong to the new class of drugs blocking effects of tumour necrosis factor A—an inflammatory mediator. Given by injection at twice weekly intervals. There are suggestions of potential for increased rate of operative infection but no consensus on whether to discontinue perioperatively.
DVT prophylaxis (p. 12): early mobilisation.
Gastrointestinal agents: continue H2 antagonists and proton pump inhibitors prior to and after surgery especially for patients on NSAIDs.
Take care of the neck and maintain in a neutral position at all times, especially on transfer and turning. Use manual in-line stabilisation during airway manipulation while the patient is unconscious (unless it is certain that the spine is stable). If intubation is necessary consider fibreoptic intubation if difficulties are anticipated ( p. 1000), particularly if there is posterior AAS (rare) and/or predicted difficulty. If direct laryngoscopy is undertaken use manual in-line stabilisation and a gum elastic bougie.
Ensure careful positioning and padding/protection of vulnerable areas on the operating table. Note comfortable position before induction, then try to maintain this during surgery.
Regional techniques may be difficult. Patient discomfort from prolonged immobilisation may favour general anaesthesia, perhaps in combination with regional techniques.
Normothermia is especially important, as hypothermia may increase the risk of wound infections.
Strict asepsis with invasive procedures as increased risk of infection.
Postoperative
Adequate pain control allows early mobilisation. PCA is often impractical due to impaired hand function. A puffer-PCA device has been described.
Continue NSAIDs unless contraindicated.
Physiotherapy and mobilisation are important.
Continue DVT prophylaxis until the patient is fully mobile.
Maintain fluid intake and monitor renal function.
Restart DMARDs to avoid exacerbation of joint immobility.
Ankylosing spondylitis
Inflammatory arthritis of the sacroiliac joints and spine, leading to ankylosis and ‘bamboo spine’. Associated with HLA B27 in >90% of cases. More common in males, with peak age onset in third decade. Important anaesthetic implications are both articular and non-articular.
Articular
Progressive kyphosis and fixation of the spine may hinder intubation. Conventional intubation and tracheostomy may be impossible. Atlantoaxial subluxation and myelopathy can occur rarely. There may be limited mouth opening from temporomandibular involvement. Use of ILMA described but fibreoptic intubation usually preferred.
At risk of occult cervical fracture with minimal trauma—ensure the head is supported and not left self-supporting.
Cricoarytenoid arthritis may make cords susceptible to trauma.
Axial skeletal involvement may make neuraxial block difficult or impossible. Spinal anaesthesia using a paramedian approach appears to be the most practical technique for neuraxial block. Possible increased risk of epidural haematoma with epidural block.
Limited chest expansion may lead to postoperative pulmonary complications. Effective external cardiac massage may be impossible.
Deformity leads to difficulty with positioning, particularly if a prone position is required.
Non-articular
Fibrosing alveolitis may occur, exacerbating postoperative hypoxia.
Aortic regurgitation (1%). Mitral valve involvement and conduction defects are rare.
Amyloid may cause renal involvement.
Cauda equina syndrome may occur in longstanding cases.
Associated use of NSAIDs and DMARDs (see p. 194).
Systemic lupus erythematosus (SLE)
This is a chronic multisystem disease commonest in young females, especially in pregnancy. It is characterised by the presence of numerous antibodies, including antinuclear antibody, and immune-mediated tissue damage. Although joints may be affected there is no deformity or bony erosion and no specific airway implications. The main anaesthetic implications are cardiovascular disease, renal disease, coagulation status, and increased risk of infection.
Preoperative assessment
Skin and joint involvement is common, as are oral and pharyngeal ulceration.
Cardiovascular: pericarditis in 15% of cases. Myocarditis and endocarditis are less common. Raynaud's phenomenon 30%. Coronary artery disease from atherosclerosis and other mechanisms common.
Respiratory system: infections and pulmonary emboli common. Pleuritis and pleural effusion. Pulmonary fibrosis less common.
Neurological: cranial and peripheral nerve lesions may occur, secondary to arteritis and ischaemia. Transverse myelitis leading to weakness or paraplegia occurs rarely. Depression, psychosis, and fits.
Renal: glomerulonephritis is a serious complication and may lead to nephrotic syndrome and renal failure.
Haematological: clotting disorders or hypercoagulable states can occur. Check FBC and clotting status. Immune thrombocytopenia or circulating anticoagulants (e.g. antibodies to factor VIII) may be present. Up to a third of patients with SLE may demonstrate features of antiphospholipid syndrome ( p. 237). This is a hypercoagulable state which paradoxically may be associated with the presence of lupus anticoagulant and a prolonged APTT. Since a prolonged APTT may indicate either a clotting disorder or a hypercoagulable state, further haematological advice should be sought.
Higher risk of stroke with antiphospholipid antibodies.
Steroids and other immunosuppressant drugs are used.
Anaesthesia
There may be absolute or relative contraindications to neuraxial blocks in patients taking anticoagulants or in patients with coagulopathy ( p. 1174). The presence of a peripheral nerve lesion may be a relative contraindication to neuraxial/regional nerve blockade.
Maintenance of normothermia may reduce the risk of infection as well as lessening the impact of Raynaud's phenomenon if present.
Laryngeal erythema and oedema are common—try to minimise trauma to the airway.
Consider hourly urine output and invasive monitoring.
Steroid supplementation ( p. 172).
Strict asepsis with invasive procedures as increased risk of infection.
Systemic sclerosis (scleroderma)
The limited cutaneous form comprising calcinosis, Raynaud's, oesophageal dysfunction, sclerodactyly, and telangiectasia (CREST) is more common (60% of cases) than the more aggressive diffuse cutaneous form, which has more widespread effects and a high mortality (see also p. 303).
The following may be relevant to anaesthesia:
Cardiovascular: Raynaud's, pericarditis, or myocardial fibrosis. Conduction defects. Pulmonary hypertension common.
Pulmonary: fibrosing alveolitis in both forms (40% in diffuse form).
Renal: may develop renal crisis associated with malignant hypertension.
Gastrointestinal: oesophageal reflux invariable.
Airway: may have mouth narrowing and tightened skin around the neck leading to difficult intubation.
No consensus on general anaesthesia vs regional.
| Rheumatoid arthritis | Ankylosing spondylitis | Systemic lupus erythematosus | Systemic sclerosis | |
|---|---|---|---|---|
Prevalence | 1% | 0.15% | 0.03% | 0.001% |
Airway and intubation | C Spine instability/ankylosis Cricoarytenoid arthritis TMJ arthritis | Cervical kyphosis, TMJ arthritis, Occult fractures | Limited mouth opening | |
Respiratory | Fibrosing alveolitis, Pleural effusions, Nodules on CXR | Fixed chest wall, Apical fibrosis (1%) | Chest infections, Pulmonary emboli, Pleuritis | Fibrosing alveolitis |
Cardiovascular | Ischaemic heart disease (association) | Aortic regurgitation 1% | Raynaud's, Hypertension, Coronary ischaemia, Pericarditis Endocarditis (Libman Sacks) | Raynaud's >90%, Hypertension, Pulmonary hypertension, Myocardial fibrosis, Arrhythmias, Pericardial effusions |
Neurological | Peripheral neuropathy, Radiculopathy, Myelopathy | Cauda equina syndrome (rare) | Peripheral neuropathy Psychosis, Convulsions | |
Renal | Mild renal impairment common | Glomerulo-nephritis | Chronic impairment Hypertensive renal crisis | |
Gastrointestinal | Drug-related gastritis | Drug-related gastritis | Abdominal pain Nausea Mesenteric vasculitis | Reflux invariable |
Haematology | Anaemia—drug-related and disease-related Felty's syndrome (splenomegaly and leucopenia) | Anaemia—drug-related and disease-related | Antiphospholipid syndrome, Anaemia, Thrombocytopenia | |
Neuraxial block | Often difficult, Infection risk | Difficult—consider lateral approach, Increased risk epidural haematoma | Check coagulation, Infection risk |
| Rheumatoid arthritis | Ankylosing spondylitis | Systemic lupus erythematosus | Systemic sclerosis | |
|---|---|---|---|---|
Prevalence | 1% | 0.15% | 0.03% | 0.001% |
Airway and intubation | C Spine instability/ankylosis Cricoarytenoid arthritis TMJ arthritis | Cervical kyphosis, TMJ arthritis, Occult fractures | Limited mouth opening | |
Respiratory | Fibrosing alveolitis, Pleural effusions, Nodules on CXR | Fixed chest wall, Apical fibrosis (1%) | Chest infections, Pulmonary emboli, Pleuritis | Fibrosing alveolitis |
Cardiovascular | Ischaemic heart disease (association) | Aortic regurgitation 1% | Raynaud's, Hypertension, Coronary ischaemia, Pericarditis Endocarditis (Libman Sacks) | Raynaud's >90%, Hypertension, Pulmonary hypertension, Myocardial fibrosis, Arrhythmias, Pericardial effusions |
Neurological | Peripheral neuropathy, Radiculopathy, Myelopathy | Cauda equina syndrome (rare) | Peripheral neuropathy Psychosis, Convulsions | |
Renal | Mild renal impairment common | Glomerulo-nephritis | Chronic impairment Hypertensive renal crisis | |
Gastrointestinal | Drug-related gastritis | Drug-related gastritis | Abdominal pain Nausea Mesenteric vasculitis | Reflux invariable |
Haematology | Anaemia—drug-related and disease-related Felty's syndrome (splenomegaly and leucopenia) | Anaemia—drug-related and disease-related | Antiphospholipid syndrome, Anaemia, Thrombocytopenia | |
Neuraxial block | Often difficult, Infection risk | Difficult—consider lateral approach, Increased risk epidural haematoma | Check coagulation, Infection risk |
Scoliosis
Progressive lateral curvature of the spine with added rotation. Scoliosis may lead to an increasing restrictive ventilatory defect which in turn leads to hypoxia, hypercarbia, and pulmonary hypertension. Corrective surgery may be carried out in the teens to arrest these changes. Scoliosis may be idiopathic (∼75%) or secondary to other conditions with anaesthetic implications:
Conduct of anaesthesia
Formal pulmonary function tests are mandatory in severe cases.
Check for pulmonary hypertension and right heart failure.
Some muscular dystrophies may be associated with cardiac abnormalities. Consider echocardiography (see p. 268).
Intraoperative spinal cord monitoring may be indicated.
Regional techniques (e.g. paravertebral blocks) where possible ± GA.
Plan for high dependency or intensive care in complex cases.
Achondroplasia
The commonest form of dwarfism is caused by premature ossification of bones combined with normal periosteal bone formation, giving a characteristic appearance of short limbs and a relatively normal cranium. The following should be noted:
The larynx may be small and intubation is occasionally difficult. Have a range of tube sizes and a difficult intubation trolley available. Laryngoscopy may be compromised by pectus carinatum.
Foramen magnum stenosis is common. Avoid hyperextension during intubation.
Central and peripheral venous access is often difficult.
Use an appropriately sized blood pressure cuff.
Obstructive sleep apnoea is common (see p. 122).
Restrictive ventilatory defects may occur and can lead to pulmonary hypertension.
Regional techniques may be difficult.
The back may be normal. The epidural space is often narrowed with spinal canal stenosis. The volume of local anaesthetic needed for an epidural is reduced.
It is difficult to predict the volume needed for a single injection spinal. Use of an incremental spinal catheter technique is suggested, but single dose spinal anaesthesia is reported. Websites giving medical advice for achondroplastics suggest that spinal anaesthesia should not be used.
The patient is of normal intelligence.
Further reading
