Autoimmune diseases cover a wide spectrum of conditions, with over 80 diseases identified so far (British Society for Immunology, 2016). Some of these affect one site only (either tissues or organs), while others have more widespread effects on multiple parts of the body, impacting the health, wellbeing and quality of life of those affected. This article is part of a two-part series and will cover some of the most well-known and common conditions, including multiple sclerosis, type 1 diabetes, rheumatoid arthritis and psoriasis, and hopes to increase practice nurses' knowledge and understanding of these troublesome diseases.
Basic pathophysiology
The function of the body's immune system is to recognise foreign elements and destroy them before they cause problems. In healthy individuals, specialised systems regulate the immune system to ensure host tissue is not attacked, and when this system functions well, the body is able to successfully defend itself against bacteria, germs and foreign bodies; however, sometimes this goes wrong and the body begins to attack its own organs or tissues. This will be explained in more detail for each condition discussed below.
Multiple sclerosis
The numbers of people affected by multiple sclerosis (MS) appears to be increasing and in 2018, there were an estimated 131 720 diagnosed cases in the UK, with women being 2–3 times more likely to be affected than men (Multiple Sclerosis Trust, 2021). The disease can occur in different forms. These are shown in Table 1.
Table 1. Forms of multiple sclerosis (MS)
| MS Type | Additional information |
|---|---|
| Relapsing-remitting | This is the most common type of MS and is associated with periods of remission and then relapse |
| Secondary progressive | Over time, progression from relapsing-remitting MS to secondary progressive MS occurs with more permanent symptoms caused by scarring of brain tissue and gradual nerve damage. Approximately two-thirds of people with relapsing-remitting MS will have developed secondary progressive MS after 15 years |
| Primary progressive | From the onset of symptoms there is a gradual worsening over time. Around 10–15% of people have this type from onset |
Pathophysiology
MS is a complex disease and, despite extensive research, its true cause remains unknown, although it is thought to be an immune-mediated inflammatory disease that attacks myelinated axons in the central nervous system (CNS), destroying the myelin and axon to variable degrees, causing significant disability in 30% of patients in a 25–30-year period (Luzzio, 2022). T cells and B cells are instrumental in causing areas of inflammation in the brain and spinal cord by attacking the myelin sheath, which surrounds nerve fibres, affecting their ability to function correctly and leading to the onset of symptoms. Environmental factors and genetic influences are thought to be a cause, but this is by no means certain (Tidy, 2020).
Signs and symptoms
Common signs and symptoms include the following (Levin, 2021):
- Paraesthesia affecting one or more extremities, the trunk or one side of the face
- Weakness or clumsiness of a leg or hand
- Visual disturbances (eg partial loss of vision and pain in one eye, double vision (diplopia))
- Urinary problems (frequency, urgency or retention)
- Gait disturbances.
Treatment
All patients with MS will be under the care of a consultant and will have the support of an MS specialist nurse. Lifestyle advice is the same as that given for other conditions and aims to encourage a healthier lifestyle. Regular exercise is thought to have benefits for people with MS and smoking cessation is particularly important as smoking is linked to the progression of disability (National Institute for Health and Care Excellence (NICE), 2020a).
Medical management
There is no cure for the disease. As many people are averse to self-injection, oral drugs are sometimes used as first-line treatments for relapsing forms of MS to increase adherence. Patient eligibility for drugs varies by which country of the UK they live in, as well as clinical factors.
The aim of medication offered is (Levin, 2021):
- Shortening acute exacerbations
- Decreasing frequency of exacerbations
- Relieving symptoms
- Delaying disability and maintaining the patient's ability to stay mobile.
Table 2 gives information on medications that may be given with additional information.
Table 2. Medical management of multiple sclerosis
| MS type | Drug treatment | Additional information |
|---|---|---|
| Relapsing-remitting | Disease modifying drugs | Interferon beta and glatiramer are established drugs for use in active relapsing-remitting MSPeginterferon beta-1 is an alternative medication and requires less frequent administrationTeriflunomide and dimethyl fumarate are options for patients with active disease and may be preferred by some patients as they are given orally. There is insufficient evidence for the use of either drug to treat highly active or rapidly-evolving severe MSFingolimod is also given orally and is the recommended treatment for patients with highly active diseaseOfatumumab is an example of a newer once per month self-injectable disease modifying drug and ocrelizumab is an example of a drug delivered by an infusion in hospital every 6 months |
| Secondary progressive | Interferon beta 1bSiponimod | Reduces the risk of relapse and short-term relapse related disability but does not prevent the development of permanent disability or slow progression once presentA sphingosine-1-phosphate receptor modulator that is an option for treating secondary progressive multiple sclerosis with evidence of active disease |
| Primary progressive | Interferon beta | There are no effective disease modifying drugs but interferon beta has been used (unlicensed). There is limited evidence to support its use because of a lack of evidence that it has an impact on disability progression |
British National Formulary, 2019; National Institute for Health and Care Excellence, 2020b
Other non-pharmacological treatments to ease symptoms include the following (Tidy, 2020):
- Physiotherapy
- Occupational therapy
- Speech therapy
- Specialist nurse advice and support
- Psychological therapies
- Counselling.
Type 1 diabetes
While type 2 diabetes accounts for around 90% of cases of diabetes, type 1 diabetes is much less common, accounting for only 8% (Diabetes UK, 2021). Type 1 diabetes can occur at any age, but onset is more common in children and young adults, with symptoms developing over days or weeks (Knott, 2021). The condition is associated with many long-term complications and requires lifelong treatment.
Pathophysiology
Type 1 diabetes is a chronic autoimmune disease characterised by the body's inability to produce insulin because of the autoimmune destruction of the beta cells in the pancreas (Khardori, 2022). In a healthy person, insulin is produced by the cells of the islets of Langerhans in response to glucose and if the body has sufficient energy, any excess glucose is converted to glycogen and stored in the liver until required. Lymphocytic infiltration and destruction of insulin-secreting beta cells leads to a decline in beta-cell mass and declining insulin secretion, until the available insulin is no longer adequate to maintain normal blood glucose levels and after 80–90% of the beta cells are destroyed, hyperglycaemia develops (Khardori, 2022). The destruction of insulin-producing cells leads to a complete absence of insulin, requiring lifelong insulin treatment.
Signs and symptoms
Signs and symptoms of type 1 diabetes include (NICE, 2020c):
- Excessive urination (polyuria)
- Excessive thirst (polydipsia)
- Rapid weight loss
- Excessive tiredness
- Random glucose of more than 11 mmol/l.
If diabetes is suspected, urgent referral is needed for the diagnosis to be confirmed.
Treatment
The management of type 1 diabetes requires intensive input and support from the diabetes team, with the addition of input from primary care by suitably qualified staff. Insulin therapy by injection is the only option and is available in varying forms. These types of insulin vary in onset and duration of action. Insulin must be tailored to meet the needs of the individual. It is beyond the scope of this article to discuss this in detail, but further information can be found in the further reading box at the end of this article.
Rheumatoid arthritis
Rheumatoid arthritis (RA) can occur at any age, but onset is most common between the ages of 35 and 50 years of age and it affects 2–3 times more women than men (Kontzias, 2020).
Pathophysiology
RA is a chronic systemic autoimmune disease that primarily affects the lining of the synovial joints and is associated with progressive disability, premature death and has a huge impact on health economies (Guo et al, 2018). The underlying cause remains poorly understood, but it is thought that an external trigger, such as infection, trauma or cigarette smoking, sets off an autoimmune reaction, which then leads to thickening of the membrane (synovial hypertrophy) lining the affected joint, leading to chronic joint inflammation (Smith, 2022). B cells produce autoantibodies (ie rheumatoid factors) and there is abnormal production of several inflammatory mediators, such as tumour necrosis factor alpha (TNF-α) and interleukin (IL)-1, as well as several others (Smith, 2022). Prior to the onset of symptoms, there is a period of pre-RA categorised by several phases. These are shown in Table 3.
Table 3. Phases of pre-rheumatoid arthritis (RA) prior to established RA
| Phase of pre-RA | Additional information |
|---|---|
| Phase I | Interaction between genetic and environmental risk factors for RA |
| Phase II | Production of RA antibodies such as rheumatoid factor |
| Phase III | Development of joint pain (arthralgia) or joint stiffness but with no clinical evidence of arthritis |
| Phase IV | Development of arthritis in one or two joints |
| Phase V | Established RA |
Signs and symptoms
Patients usually present with symptoms of pain and stiffness, which is often worse on waking. Morning stiffness of the affected joints may last for more than 30 minutes and in addition the patient may experience fatigue, fever, weight loss, joints that are tender, swollen and warm, and have rheumatoid nodules under the skin (Bullock et al, 2018). Although the disease course varies from patient to patient, generally progression is most rapid during the first 6 years, particularly the first year, with 80% of patients developing some permanent joint abnormalities within 10 years (Kontzias, 2020).
Treatment
Treatment is initiated in the secondary care setting and aims to minimise pain and reduce damage to the joints, reduce disability and improve quality of life. Options are as follows (NICE, 2020d):
- A disease modifying drug (DMARD) as monotherapy (eg methotrexate, leflunomide or sulfasalazine), ideally commenced within 3 months of symptom onset
- Hydroxychloroquine may be used for patients with a relapsing-remitting type of RA (palindromic disease)
- Short-term glucocorticoids may be given (either orally, intramuscularly or intra-articularly) to improve symptoms while the patient waits for the DMARD prescribed to take effect
- Step up treatment may involve the addition of further DMARDs
- For patients with severe disease, biologic agents may be given, either alone or in combination with methotrexate. Readers can refer to suggested further reading for more information.
All DMARDs require regular blood testing, which may be done in primary care under a shared care arrangement. Information on bloods needed for the most common drugs prescribed, and their frequency, is shown in Table 4.
Table 4. Monitoring of bloods for commonly prescribed disease-modifying antirheumatic drugs (DMARDs)
| Drug | Blood test required | Frequency |
|---|---|---|
| Methotrexate |
|
Every 2 weeks until dose is stable, then monthly for 3 monthsThereafter at least every 12 weeks but may need to be more frequent in patients at risk of toxicity |
| Leflunomide |
|
Monitoring is same as for methotrexate, but if dose is increased, bloods should be taken every 2 weeks until dose is stable and regime can then revert to previous schedule |
| Sulfasalazine |
|
Same as for leflunomide but monitoring can be stopped after 12 months |
| Hydroxychloroquine | None | No routine bloods are required but an eye test is advised when commencing the drug and annually for those taking it long term (5 years or more) |
Psoriasis
Psoriasis is a chronic inflammatory skin condition, which occurs most frequently in the 15–25 and 50–60 age groups (Thomson, 2021). The rash has a scaly appearance and can be mild or more severe, appearing at more than one site. There are several forms of psoriasis; plaque psoriasis being the most common accounting for 80–90% of cases (NICE, 2021). Other less common types are shown in Table 5.
Table 5. Less common psoriasis types
| Psoriasis type | Additional information |
|---|---|
| Localised pustular | This is the second most common type and typically affects the soles of the feet and the palms of the hands |
| Guttate | Can occur anywhere on the body and accounts for 2% of psoriasis cases |
| Flexural | Affects approximately 3–7 % of people with psoriasis. Occurs at sites such as the armpits, under the breasts or the groin |
| Generalised pustular | Potentially life-threatening medical emergency with a rapidly spreading rash, which is associated with fever, tachycardia, malaise and arthralgia |
| Erythrodermic | Very rare. Widespread severe psoriasis, which is potentially life threatening |
| Nail | Present in about 50% of patients at diagnosis and is particularly common in patients with psoriatic arthritis (up to 90% have nail involvement) |
Pathophysiology
Psoriasis is a chronic condition with a complex underlying pathology, and is thought to be influenced by genetic, environmental and immunological factors (Thomson, 2021). Recent research has suggested that psoriasis is an autoimmune disease, with abnormally large numbers of T-cells triggering the release of cytokines in the skin, causing the inflammation, redness, itching and flaky skin patches characteristic of the condition (Primary Care Dermatology Society, 2022). Genetic factors are also important, with family history present in 40–50% of cases, rising to as much as 75% if onset occurs before the age of 20 years (Primary Care Dermatology Society, 2022).
Treatment
Emollients play an important part in treating psoriasis and these are available in various forms. Ointments tend to be greasier, creams tend to be less so, but whichever choice the patient makes, they should use their emollients frequently. Moisturising the skin helps to soften plaques, remove scales and prevent itching and should be applied 30 minutes before applying other treatments to allow absorption (Payne, 2018). Other treatment options include (Payne, 2018):
- Vitamin D-based treatments: available as creams, lotions, ointments and also in a scalp formation. Can be prescribed as a combination product containing a steroid (eg calcipotriol/betamethasone), often useful at onset of a flare up
- Steroid creams or ointments: these are commonly used to reduce skin inflammation. Examples include hydrocortisone, clobetasone butyrate (eg Eumovate) and betamethasone (eg Betnovate)
- Coal tar: several preparations are available for scalp psoriasis (eg T/Gel shampoo, Psoriderm scalp lotion) and also creams for psoriasis on the trunk (eg Evorex, Psoriderm). These are unsuitable for use in the first 3 months of pregnancy. Can have an unpleasant smell and stain clothes/bedding
- Dithranol: for many people, a daily application to a plaque will resolve it, but may take weeks to do so. However, it is messy and stains skin, hair, bedding and clothes, and can irritate healthy skin
- Salicylic acid: often combined with other treatments and if used prior to steroid creams or coal tar, is effective in loosening scales and making other treatments more effective
- Tazarotene: vitamin A-based preparation sometimes used. Can irritate healthy skin and is not suitable for pregnant or breastfeeding women.
Trigger factors
Treatment aims to settle the rash but there is no complete cure and many will experience intermittent flare ups throughout their lifetime. There is often no reason why these occur but a number of factors have been highlighted as possible triggers. These are (Payne, 2018):
- Smoking
- Alcohol
- Stress
- Hormonal changes
- Sunlight
- Trauma
- Infection
- Obesity
- Certain medications.
Conclusion
There are many autoimmune diseases and each has different underlying pathology, symptoms and treatment, and a varying impact on the individual affected. They are complex and difficult to understand and it is hoped that this article gives practice nurses and non-medical prescribers an increased understanding of the diseases discussed and will help them when giving advice to patients coping with any of these conditions.
Further reading:
- MS Trust. Multiple sclerosis information for health and social care professionals. 2022. https://mstrust.org.uk/health-professionals/health-professional-resources
- National Institute for Health and Care Excellence. Insulin therapy in type 1 diabetes. 2020. https://cks.nice.org.uk/topics/insulin-therapy-in-type-1-diabetes/
- GP online. Rheumatoid arthritis: the basics. 2022. https://www.gponline.com/basics-rheumatoid-arthritis/rheumatology/rheumatology/article/855412
- Kim WB. Diagnosis and management of psoriasis. 2017. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5389757/
KEY POINTS:
- Autoimmune diseases cover a wide spectrum of conditions with over 80 diseases identified so far
- Sometimes the immune system goes wrong and the body begins to attack its own organs or tissues
- Multiple sclerosis, type 1 diabetes, rheumatoid arthritis and psoriasis are some of examples of the most well known and common autoimmune diseases
CPD reflective practice:
- What is the difference between the immune system of healthy individuals and those with an autoimmune disease?
- What lifestyle advice could you give to patients with multiple sclerosis, or other types of autoimmune disease?
- Can you describe some of the trigger factors for a flare up of psoriasis?