11.1 Rheumatoid Arthritis

EXPLANATION OF CONDITION

Rheumatoid arthritis (RA) arises when IgM autoantibodies (rheumatoid factor) activate the complement system to inflame the synovial membrane of joints, tendons, bursae and often the pericardium of the heart. Inflammation is accompanied by an influx of inflammatory cytokines, including tumor necrosis factor (TNF) alpha³, which induce synovial membrane to thicken (hyperplasia) and be thrown into folds (pannus) with a subsequent increase of synovial fluid, causing painful swelling and restricted movement. Cells of the synovial membrane (synoviocytes) invade the joint cartilage where they secrete enzymes initiating destructive changes in bone and cartilage^3,4.

Finger and toe joints are usually affected first with subsequent involvement of larger synovial joints. Onset can be chronic, over several weeks, with early morning stiffness, swelling of joints and progressive joint pain. Alternatively onset can be acute, with fever and generalised illness. On occasion a palindromic pattern might present with pain and inflammation appearing to ‘flit’ from one joint to another, which can be misdiagnosed as more trivial aches and pains. There are usually periods of remission and relapses⁵. Tiredness results with a temptation to snack on carbohydrate foods and avoid exertion, with the inherent risk of obesity⁶.

RA is three times more common in women and the incidence increases with age. It can occur in children as juvenile idiopathic arthritis (JIA) or juvenile rheumatoid arthritis (JRH).

There is a genetic predisposition in around 50% of cases^7,8, and infection is implicated in 10-20% cases^7–9 Risk factors include winter, smoking, obesity, blood transfusion and pet ownership⁷. The oral contraceptive pill may offer protection or delay the onset¹⁰. Whilst it is rare for RA to present in pregnancy¹¹ onset is associated with the puerperium, particularly in women who breast-feed after their first delivery⁹.

Diagnosis is made by clinical examination and laboratory results showing raised erythrocyte sedimentation rate (ESR) or inflammatory markers, and a positive rheumatoid factor in some cases⁶. Prognostic criteria are under review¹² but currently, with modern management, 20% have mild disease, 75% moderate disease with relapses and remissions and 5% have severe destructive disease⁵.

COMPLICATIONS

• Reduced movement of affected joints
  
• Wasting of small muscles of the hand
  
• Osteoporosis and fractures⁵
  
• Swelling of soft tissue around the joints
  
• Ruptured tendons or joints (Baker’s cysts)
  
• Spinal cord compression
  
• Neuropathy
  
• Infection (secondary to steroids)
  
• Secondary anaemia
  
• Sjögren’s syndrome (dry eyes)
  
• Secondary Raynaud’s phenomenon (see Section 11.2)
  
• Scleroderma (see Section 11.2)
  
• Lungs develop pleural effusion and nodules
  
• Heart develops asymptomatic pericarditis
  
• Reduced life expectancy from cardiac complications⁵

When JIA was followed up in adulthood¹³, 83% of those affected were sexually active, but affected by:

• Relationship instability and sexual problems
  
• Detrimental effect on body image
  
• Short stature (associated with caesarean section)
  
• Reduced hip mobility (associated with caesarean section)

NON-PREGNANCY TREATMENT AND CARE

Modern management advocates disease-modifying anti-rheumatic drugs (DMARDs) used at disease onset¹⁴ to control disease activity and reduce cardiovascular mortality¹⁵ with monitoring for side effects⁵. TNF inhibitors can be started after a 6-month trial on two DMARDs if the condition worsens⁵. Examples of drugs include:

• NSAIDs to control symptoms
  
  
  
  • aspirin
    
  • indometacin (Indomod)
    
  • ibuprofen (Brufen, Nurofen)
    
  • naproxen (Naprosyn)
    
  • phenylacetic acid (Diclofenac)
    
  • ketoprofen (Ketocid)
  
  
• Steroids to reduce inflammation
  
  
  
  • prednisolone (Deltacortril)
  
  
• DMARDs to modify disease
  
  
  
  • azathioprine (Imuran)
    
  • ciclosporine (Neoral)
    
  • gold (Myocrisin, Ridaura)
    
  • hydroxychloroquine (Plaquenil)
    
  • leflunomide (Arava)
    
  • methotrexate (Maxtrex)
    
  • penicillamine (Distamine)
    
  • sulfasalazine (Salazopyrin)
  
  
• TNF inhibitors to suppress the immune response
  
  
  
  • etanercept (Enbrel)
    
  • infliximab (Remicade)
  
  
• Contraception is essential as fertility is normal and some of the above drugs are contraindicated in pregnancy¹⁶
  
• Diet: specific diets are not supported by evidence and fasting followed by a vegetarian diet risks malnutrition¹⁷
  
• Physiotherapy
  
  
  
  • assessment, with the provision of aids to enhance mobility
    
  • exercises and/or hydrotherapy to maintain joint function and muscle power
    
  • splints to support joints during disease flare

PRE-CONCEPTION ISSUES AND CARE

Re-refer to the rheumatologist for a risk benefit analysis to maintain or alter drug therapy, ideally in consultation with a consultant in maternal medicine. Azathioprine, hydroxychloroquine and sulfasalazine might be continued. Other DMARDs can be replaced by steroids¹⁸. Paracetamol for pain relief³.

Avoid methotrexate¹⁸. Leflunomide should be stopped, with effective contraceptive cover, for 2 years prior to conception or until plasma levels fall below 0.02 mg/l¹⁸ (see Appendix 11.1.1).

Folic acid 0.4 mg is important, as folate deficiency results from long-term DMARD usage¹⁹.

Assess weight; if necessary implement obesity reduction measures. Encourage a vitamin- and iron-rich diet (see Appendix 1.2). Promote smoking cessation, and regular exercise.

11.2 Raynaud’s Phenomenon

EXPLANATION OF CONDITION

Maurice Raynaud first described a cold-induced transient, painful cessation of blood flow to the fingers and toes with triphasic colour changes in 1862⁶. The peripheral blood circulation becomes congested following arteriolar spasm under sympathetic nervous control on exposure to a cold temperature or stress⁷. Attacks usually subside after a few minutes. The middle fingers are most commonly affected, but not the thumb⁸, and sometimes toes, ears, and the tip of the nose. Colour changes comprise^6,7:

• White – becomes painful due to ischaemia
  
• Blue – becomes numb due to cyanosis
  
• Red – becomes painful when reperfusion of blood occurs

The condition is probably under-recognised, as only 2% of sufferers consult a doctor⁴and many try natural remedies before conventional treatment⁹. Of unknown aetiology¹⁰, there are varying associations with cigarette and alcohol consumption^11,12 and a strong correlation with anxiety traits¹³. There are two types: primary and secondary.

Primary Raynaud’s Phenomenon (PRP) (90% of cases²)

• Young women mainly affected, onset commonly at puberty^1,2
  
• Can affect children, even in early childhood¹⁴, with a female predominence^15,16
  
• Familial^17,18 in 26% of cases¹⁹
  
• Migraine headache (see Section 8.1) in 15–27% of cases^20,21
  
• Condition may go into remission^10,22
  
• If nailform and autoantibody tests are normal (90% of PRP) the condition is benign¹; reassurance and advice can be given^2,23 and the condition managed by the GP usually without drug treatment^{2, 9}
  
• If nailform and autoantibody tests are abnormal (10% of PRP) there is risk of a defined connective tissue disease developing within 10 years^2,10, especially if there was a higher age of onset²⁴, hence consultant referral is indicated

Secondary Raynaud’s Phenomenon (SRP) (10% of Cases²)

Usually presents later in life as it arises from occupational hazards or another medical condition and drug therapy. Common associations:

• Hand vibration injury (repetitive strain)⁴
  
• Carpal tunnel syndrome²⁰
  
• Beta-blocker use^1,20
  
• Systemic lupus erythematosus (SLE) in 10–45% of cases⁵
  
• Sjögren’s syndrome (dry eyes and mouth) in 33% of cases⁵
  
• Rheumatoid arthritis in 10–20% of cases⁵ (see Section 11.1)
  
• Scleroderma (systemic sclerosis, scleroma) >90% of cases⁵
  
  
  
  • literally ‘hard skin’ which becomes thick and taut with a waxy appearance due to swelling of collagen fibres and constriction of the peripheral capillary blood vessels
    
  • Renal, respiratory and gastrointestinal tracts progressively affected²⁵
    
  • 5-year survival rate in 37–74% of cases²⁶
    
  • Uncommon in nulliparous women, very rare in pregnancy, and 87% had their last pregnancy ≤5 years before the onset of scleroderma²⁵
  
  
• Other connective tissue diseases

COMPLICATIONS

Only a small number of those with primary Raynaud’s will go on to develop connective tissue disease, usually 10 years from the onset of Raynauds²⁷. Those with SRP, and the chronically sick and disabled, risk complications which include:

• Chilblains and mouth ulcers
  
• Skin rashes
  
• Livedo reticularis (red/blue skin mottling)⁷
  
• Cyanosis and ulceration of tips of fingers and toes which, with SRP, can lead to gangrene in extreme cases⁷

NB: Midwives are most likely to care for mothers with primary

Raynaud’s phenomenon and minimal complications.

NON-PREGNANCY TREATMENT AND CARE

As Raynaud’s can precede connective tissue disease, especially scleroderma¹⁰,²², investigations are required:

• Blood tests for FBC, ESR, and autoantibody screen
  
• Nailform capillary test using an ophthalmoscope⁹

NB: If these are abnormal, underlying connective tissue disease is implied, so keep under review or refer to a specialist centre⁹

Advice

• Avoid extremes of temperature²⁸
  
• Wear warm clothing²⁸
  
• Cease smoking and minimise caffeine intake²⁸
  
• Undertake regular exercise²⁸
  
• Mention this condition when seeking contraceptive advice – the combined contraceptive pill may be contraindicated
  
• Relaxation techniques if stress is a contributing factor²³

Drug Therapy (more likely with (SRP)

• Many patients try natural remedies before prescribed drugs⁹
  
• Vasodilator nifedipine (Adalat, Tenif)^9,23
  
• Calcium channel blockers²³
  
• Other treatment is dependent on the underlying condition.

NB: Put an alert note on case notes, as she should not take beta-adrenoceptor antagonists or vasoconstrictor drugs

PRE-CONCEPTION ISSUES AND CARE

Primary Raynaud’s

• Combined oral contraceptive pill might be contraindicated
  
• Risk–benefit assessment for cessation of drugs peri-conception
  
• Promote smoking cessation
  
• Advise that PRP is unlikely to affect a forthcoming pregnancy

Secondary Raynaud’s (as Above, Plus)

• Management and implications for childbearing depends upon the co-existing condition
  
• Scleroderma has functional problems with coitus²⁸ and its potential mortality may raise significant dilemmas about termination or continuation of a pregnancy
  
• If Sjögren’s syndrome symptoms are apparent investigate for anti-Ro/-La antibodies, which cross the placenta with a 10% risk of neonatal lupus syndrome (see Section 11.3)

11.3 Systemic Lupus Erythematosus

EXPLANATION OF CONDITION

With lupus erythematosus, the body produces autoantibodies against its own connective tissue. Shortened to lupus, there are several variations of which the two most common are:

Lupus has periods of remission, and when active is termed ‘lupus flare’ which is often triggered by infection, exposure to sunlight or oestrogen increase – as may occur when prescribed the oral contraceptive pill. It can be difficult to differentiate between a flare and infection².

Diagnosis is based using clinical criteria⁵ and investigations. Skin biopsy is used to detect SLE autoantibodies reacting with its complementary antigen⁶. Patients usually test positive for antinuclear antibody (ANA) and often DNA antibodies (70–80%). A raised ESR is common, and a positive rheumatoid factor is found in 25%. A false positive test for syphilis is found in 33%⁷ and some have antiphospholipid antibodies⁸, which can result in a co-existing antiphospholipid syndrome² with thrombo-embolic sequelae⁸.

There is no cure for SLE and treatment aims to control symptoms and prevent progression. With modern treatment the survival rate has increased to 15 years in 80% of cases⁹. Death usually results from generalised disease, infection or cardiovascular disease.

COMPLICATIONS

Some of the following manifestations may be experienced^2,8:

• Acute and chronic infection
  
• Nausea, vomiting and diarrhoea
  
• Alopecia (hair loss)
  
• Photosensitivity
  
• Arthritis and sometimes early morning stiffness
  
• SRP (see Section 11.2)
  
• Sjögren’s syndrome (dry eyes/mouth) (see Section 11.2)
  
• Ulceration of mouth, nose and vagina
  
• Fatigue and myalgia (muscle pain)
  
• Jaccoud’s arthropathy (deformed joints due to lax ligaments)
  
• Renal disease (lupus nephritis) in 40–75% of cases
  
• Pleurisy (often asymptomatic)
  
• Ischaemic heart disease
  
• Pulmonary hypertension
  
• Pericarditis (often asymptomatic)
  
• Anaemia – as a result of chronic infections⁶
  
• Leucopenia (reduced leucocyte count)
  
• Thrombocytopenia
  
• Neuro-psychiatric states, e.g. cerebrovascular accident², migraine, epilepsy¹⁰ and depressive or manic symptoms¹¹ and dementia¹⁰

NON-PREGNANCY TREATMENT AND CARE

Advice for the Woman

• Avoid sunlight and use sun block
  
• Avoid infection situations
  
• Avoid unplanned pregnancy and seek pre-conception care
  
• Avoid stress and get adequate rest (may need to adapt job)
  
• Use analgesics as required
  
• Protect against cold if Raynaud’s phenomenon occurs
  
• Eat a well-balanced diet
  
• Have a positive self-image (e.g. camouflage make-up)

Monitor

• Urinalysis for blood and protein (indicates renal disease)
  
• Regular blood pressure measurement
  
• Screening for diabetes
  
• Assessing osteoporosis risk (from prolonged steroid use)
  
• Investigations for antiphospholipid (Hughes) syndrome
  
• Blood tests for FBC, ESR, WBC, U&E, creatinine, C3 and C4 complement and anti-DNA titre^5,8
  
• LFT if taking azathioprine

Medical Treatment

• Corticosteroids for disease flare, e.g. prednisolone⁷
  
• NSAIDs for pain, fever and arthritis⁷
  
• DMARDs to arrest disease progression
  
  
  
  • methotrexate
    
  • cyclophosphamide²
    
  • azathioprine
    
  • mycophenolate
  
  
• Antimalarial drugs (e.g. hydroxychloroquine) for skin disease, fatigue and arthralgia^7,8

PRE-CONCEPTION ISSUES AND CARE

• Pre-conception counselling is essential to allow accurate assessment of the woman’s disease, permitting discussion of the potential complications of pregnancy – including pre-eclampsia, intra-uterine growth restriction and premature birth
  
• Refer to obstetrician and rheumatologist with specialist expertise in SLE/pregnancy to facilitate the above and review current medication and adjust as required
  
• Advise conception during a period of disease remission as this decreases the risk of pregnancy complications. SLE does not appear to affect fertility¹² so contraceptive advice is important
  
• Women with active lupus nephritis at conception have a higher risk of decreasing renal function during pregnancy which may occasionally be permanent¹³

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