Pathophysiology of Systemic Lupus Erythematosus

Introduction

This essay will explore the pathophysiology relating to a call I attended while on practice placement as a student technician. The initial call details were that it was a 45 year old female in pain. On our arrival the patient was sat in a chair and was breathing rapidly and had a very flushed face. After introducing ourselves and gaining the patients consent we then proceeded to take her basic observations and take her history. Most of her observations were within acceptable limits however her blood pressure was slightly high, she had a raised pulse at 120 beats per minute and slightly high temperature at 38 degrees Celsius. On questioning the patient had a localised pain score of 8/10 in her back and a pains in her joints and muscles with a pain score of 4/10. When questioned about her medical history, she stated she had a condition called systemic lupus erythematosus. This is not a condition I had heard of before I, therefore, questioned her more about it.

Differential Diagnosis

A 20 year-old women patient presented with fatigue, heart palpitations and anxiety. Reviewing the patient’s history revealed that she started experiencing anxiety, fatigue and heart palpitations approximately two years ago when she was still pregnant and she arranged to visit her own doctor. At that time her doctor stated that this was was due to stress. However, her symptoms became worse after she had another child. She stated that before going into labour she experienced premature contractions, headaches, increased fatigue, and swelling of her legs. Her doctor advised her to stay off work and have some bed rest. Shortly after giving birth to her third child, the patient began experiencing several new symptoms. Her knees and ankles started swelling, she also developed sever pain in her knees, wrists, elbows, and fingers. During the winter the patient started feeling painful and her peripheral joints became discolored whenever exposed to cold. She had recently been admitted to hospital in order for tests to be carried out and although she was still waiting for a definative outcome, a rhueamatology consultant she was under stated he was fairly certain that she had stemic lupus erythematosus.

Epidemiology

According to Somers et al (2007) approximately five people in every ten thousand suffer from systemic lupus erythematosus. Ninety percent of patients who are diagnosed with systemic lupus erythematosus are female (Ginzler&Tayar 2012; Blank et al 2009). It most commonly develops in women aged between 20 and 40 although anyone of any gender and age can develop it at any time. With regards to ethnic origin systemic lupus erythematosus is more prevalent in those with Afro-caribean, Asian or Chinese ancestry. Although there is a possibility of having a genetic pre-disposition to the disease only 3 of 100 children of those diagnosed with systemic lupus erythematosus will go on to develop it. According to National Health and Nutrition Examination Survey (2004) a survey in which it was observed that 100% of patients with System lupus erythematosus were receiving treatment.

Aetiology

Although there is no definate cause of systemic lupus erythematosus (Giannouli 2006), there is thought to be several factors increasing the likelihood of developing systemic lupus erythematous they include Genetics {where members of the family have lupus this condition will often increase the child’s chances of developing it} (Hemminki 2009). It is also thought it can also be triggered by environmental factors such as trauma, ultraviolet rays and also emotional stress. Gender and hormones is thought to be the biggest factor affecting the chances of developing systemic lupus erythematous as it is proven that it mostly affects women than men especially when they are in menstrual periods or in their pregnancy, it is said to occur nine times more to women in comparison to men (Ginzler&Tayar 2012).

Pathophysiology

System lupus erythematosus is an autoimmune disease. The autoimmune system is responsible for fighting foreign and often harmful bacteria in the human system. However with systemic lupus erythematosus the immune system will attack itself, the pathology of this disease is similar to that of rheumatoid arthritis and can affect any part of the body (Ginzler&Tayar 2012). Systemic lupus erythematosus may also occur in the form of periarticular inflammation that affects the tendon sheaths, the illness causes the patient to experience coexisting fibromyalgia that results to poor sleep, chronic disease, inactivity ,mood problems and depression (Knott 2012).

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Patients who suffer from this condition have abnormalities when it comes to their immune system, this includes the B cell function, apoptosis and also the T cell function. Systemic lupus erythematous mostly attacks the blood vessels, the skin, the heart, nervous system, liver, lungs and the kidneys (Knott 2012). Unfortunately when System lupus erythematous persists without effective treatment it may result in serious complications such as stroke, heart inflammation, lung damage, blood clots and if the patient is female it may result in miscarriage or pregnancy complications. Research shows that the exact cause of immune deregulation is not yet clear but it may be as a result of interference of endogenous metabolism that affects the function of antigens in the human system (Ginzler&Tayar 2012).

The abnormalities caused by System lupus erythematosus may vary from one ethnic group to another for instance East Asians who have this problem they have extra cytotoxic T lymphocyte antigen-4 while the white populations abnormalities is observed through having more Fc-Y receptors (Hemminki 2009) . For effective diagnosis of System lupus erythematosus it is of essence to deal with specific autoantibodies in order to treat the root cause of the disease (Ginzler&Tayar 2012).

The symptoms of systemic lupus erythematosus will vary from patient to patient as the signs and symptoms displayed will depend on the body part or system that is being affected by the systemic lupus erythematosus (Arthritis Research UK 2014). However there are some generalised symptoms and these are about to be discussed.

Dermatological symptoms caused by systemic lupus erythematosus could include rashes and in some cases a specific rash to the cheeks known as a “butterfly rash” can be identified (Knott 2012). Another common issue for the majority of people with systemic lupus erythematosus find that they become much more sensitive to sunlight. It is also common for the blood vessels under the skin in the joints to become inflamed and this can lead to poor circulation to these areas (this is called Raynauds phenomenon)(Ginzler&Tayar 2012). Oral ulceration can sometimes occur for this very reason.

Blood disorders have been known to develop especially in children, the most common being anaemia. Having anaemia would obviously cause lethargy and low mood (Giannouli 2006).

Patients with systemic lupus erythematosus are much more at risk of developing cardiac issues than the general public as the disease often causes inflammation of the blood vessels within the heart, thus increasing the risk of developing atherosclerosis which in turn could lead to a myocardial infarction if one of the blood vessels was to become blocked(Blank et al 2009).

If the kidneys were to become inflamed due to the systemic lupus erythematosus, this could cause symptoms including haematuria (blood in the urine). Also, if the kidneys become inflamed this could lead to the patient developing a kidney infection which will result in the patient having moderate to severe back pain (Ginzler&Tayar 2012).

The most common issue for patients suffering from systemic lupus erythematosus is to do with their joints (Somers et al 2007). Most people suffering from systemic lupus erythematosus will develop pain in their muscles, joints and peripheral joints such as joints in their hands and knees can often become inflamed. This can be due to a condition known as Osteonecrosis developing due to the systemic lupus erythematosus (Giannouli 2006). Osteonecrosis is a condition that develops when there is a reduced blood supply to the bones. Osteonecrosis will affect the bones ability to replace old bone with new bone and if an injury is sustained it may never be able to heal properly (Knott 2012).

Most patients find that it is worse when they wake up in the morning and as this is a chronic condition, it can often feel better in periods of remission and more painful during a relapse period (Ginzler&Tayar 2012).

Diagnosis

The process involves the blood tests including antibody tests, urinalysis and a chest X-ray which is mostly overseen by Rheumatology Consultants whose area of specialisation is autoimmune diseases and also soft tissue treatment. Musculoskeletal symptoms are analysed when trying to diagnose System lupus erythematous and it can be manifested as arthritis or arthralgia in which the patient expresses it as stiffness and pain, when it occurs in System lupus erythematous it can be in the form of migratory or transient pain and it is difficult to be diagnosed since it may be present when the patient makes the appointment with a doctor only to be resolved in the process of evaluation(Ginzler & Tayar 2012 and Knott 2012). Systemic lupus erythematosus caused fewer erosions or fixed deformities unlike rheumatoid arthritis which is much more degernerative to the joints (Ginzler&Tayar 2012). There is also the possibility of around 4% of the patients will suffer from myositis ( which is the inflamation of the muscle tissue) and this can be examined by taking a biopsy of muscle tissue (Giannouli 2006).

Pharmacology and associated treatment:

Unfortunately there is no cure for Systemic lupus erythematosus and the treatment plan is based on symptom relief as opposed to direct treatment of the disease(Ginzler&Tayar 2012, Blank et al 2007, Knott 2012). Treatment of Systemic lupus erythematosus is very specific to the individual patient and it always depends in manifestation of the symptoms, the disease severity and most importantly the specific organ affected(Giannouli 2006). In order to devise the most appropriate treatment plan the rheumatology consultant will perform a number of diagnostic tests and depending on what the results are will influence the treatment plan (Giannouli 2006).

Patients suffering from systemic lupus erythematosus will often need to take a group of medications called non-steroidal anti inflammatory drugs (Knott 2012). This group of medications work by reducing the inflammation in the affected joints and hopefully reducing the amount of pain the patient is in. Some common non-steroidal anti inflammatory drugs are ibuprofen, aspirin and diclofenac sodium (these are all available without a prescription from a pharmacy), the rheumatology consultant may recommend Naproxen or pirixicam in certain circumstances (BNF 2014). With non-steroidal anti inflammatory drugs there is a risk that they may cause damage to the lining of the stomach and could even cause ulceration in the stomach when taken over long periods of time in high doses(Knott 2012).

If the patient is taking frequent non-steroidal anti inflammatory drugs they may be prescribed a proton pump inhibitor medication in order to protect the stomach. These medications work by reducing the amount of acid the stomach produced and thus protecting the lining of the stomach (Knott 2012). Common proton pump inhibitor medications include; omperazole, lansoprazole, pantoprazole and esomeprazole (BNF 2014).

A further treratment that a rheumatology consultant may consider is the use of a medication called hydroxychloroquine (Giannouli 2006). Hydroxychloroquine was originally an anti-malarial drug however it has recently started being used to treat the symptoms of systemic lupus erythematosus and is a disease modifying anti-rheumatic drug (also known as DMARD)(Knott 2012). Hydroxychloroquine works by reducing the response from the immune system which is causing the symptoms (this is probably the closest treatment to treating the cause as opposed to just the symptoms). It is used as a long term treatment to try and prevent relapses or flare ups of the disease (Ginzler&Tayar 2012).

Corticosteroids may be used in severe systemic lupus erythematosus (Ginzler&Tayar 2012). They may be prescribed during relapses of the disease. Corticosteroids work by blocking the normal function of the white blood cells and reducing the inflammation response(Knott 2012). However the negative aspect of this is that it leaves patients very vulnerable to infections(Ginzler&Tayar 2012). Other side effect could include the patients bones becoming more fragile, the skin becoming thinner, hypertension and also weight gain. For this reason corticosteroids will only be used to treat flare ups at the minimum effective dose and then reduce the dose slowly as the symptoms ease. Corticosteroids used in the treatment of this disease are hydrocortisone and predniselone(BNF 2014).

Often used in conjunction with corticosteroids during a relapse are a group of medications called immunosuppressants . These medications will work by reducing the action of the immune system and will be used when the immune system is attacking a healthy part of the patients body(Knott 2012). Again these medications will reduce the pateints ability to fight harmful infections and can cause many side effects. Immunosuppresent medications used include: azathioprine, mycophenolate mofetil and cyclophosphamide (BNF 2014).

The final medication to be discussed is called Rituximab. Rituximab was originally created to treat blood cancer however an “off-label” use for this medication is to treat autoimmune diseases. The way Rituximab works is by destroying the B-Cells(The B-Cells which cause the release of antibodies resulting in the symptoms). As with all the other medications there could be side effects the most common being dizziness and vomiting. Rituximab can only be administered intravenoulsy so will normally require hospital admission for the patient to receive it .

(Knott 2012)

Other interventions include an advice session from the doctor regarding the patients lifestyle and diet may be of vital importance this may help the patient and minimise lifestyle related stress (Ginzler&Tayar 2012) . The patient may be suggested to work with a support group or even receive counselling as the condition can cause severe and life limiting symptoms while in relapse. Most patients with systemic lupus erythematosus are also advised to avoid the sun as it can aggravate and skin symptoms(Knott 2012).

Conclusion

Systemic Lupus Erythematosus is a very complex disease and can present itself in many different ways depending on the patient and the part of the body affected. In the pre-hospital environment it is very important to get a detailed history in order to identify whether they have been diagnosed with this disease. Clinicians must remember systemic lupus ertythematosus can limit a patients quality off life while they are having a relapse so it is important to assess each patients individual symptoms and if necessary administer pain relief and position them so they are comfortable. It is also important for ambulance clinicians to be mindful of the patients joints when using moving and handling techniques as this could obviously cause the patient discomfort or even pain due to the condition.

References:

Arthritus Research UK. (2014). Systemic Lupus Erythematosus. Available: http://www.arthritisresearchuk.org/arthritis-information/drugs/hydroxychloroquine/what-it-is.aspx. Last accessed 20/03/2014.

Blank M, Shoenfeld Y, Perl A. 2009. Cross-talk of the environment with the host genome and the immune system through endogenous retroviruses in systemic lupus erythematosus. Lupus. Nov;18 (13):1136-43

Giannouli, S (2006) Annals of rheumatic disease, Anaemia in systemic lupus erythematosus from pathophysiology to clinical diagnosis. 65(2) p144-148

Ginzler E, Tayar J. 2012. Systemic lupus erythematosus (lupus). Updated: January 2012. Available at http://www.rheumatology.org/practice/clinical/patients/diseases_and_conditions/lupus.pdf#search=sle. [Accessed on February 21, 2014 at 21:30]

Hemminki K, Li X, Sundquist J, Sundquist K (February 2009). “Familial associations of rheumatoid arthritis with autoimmune diseases and related conditions”. Arthritis Rheumatology. 60 (3): 661–8

Knott, L. (2012). Systemic Lupus Erythematosus. Available: http://www.patient.co.uk/health/systemic-lupus-erythematosus. Last accessed 20/03/2014.

Somers E, Thomas L, Smeeth L .2007. Incidence of systemic lupus erythematosus in the United Kingdom, Arthritus Rheum 15;57(4) p612 – p618.