Complications Of Juvenile Idiopathic Arthritis: A Case Overview

Summary

I saw a 61-year-old lady on the orthopaedics ward during my MDEMO placement. She had been admitted on the 9/4/19 as a result of worsening pressure sores around her sacral region. The patient used an electric wheelchair and had a number of co-morbidities, resulting from Juvenile Idiopathic Arthritis (JIA), diagnosed when she was at ten years old. I visited her again on the 30/4/2019 and she was being treated using a Vacuum-assisted closure to help with the healing of the sores. She was also being nursed in isolation whilst being investigated for a potential MRSA infection. I am describing this case to discuss the complications of JIA.

Background

This case highlights the difficulties associated with managing chronic illness and the long-term comorbidities that can develop. It demonstrates how JIA can have a significant impact on a person’s health. I will discuss the clinical features of this disease, the multimodal treatments available and the importance of allied health professional input into patients with complex needs. The impact of the newer biological agents on the long-term burden of this disease will also be discussed.

Case Presentation

This lady was admitted with worsening pressure sores and a painful, chronically dislocated, right hip. This lady used an electric wheelchair and had ceiling hoists in place at home to facilitate transfers into her bed and bath. Over the previous month, her hip had become increasingly stiff, making it difficult to mobilize and eventually impossible to eat unaided, as the motion of bending forwards became too painful. During this time, her existing chronic pressure sores deteriorated, adding to her pain, eventually leading her to call for an ambulance.

This lady first experienced pain in her ankles when she was seven-years-old. This made it increasingly difficult to walk to school, until she had to be taken to school in a pushchair. She was later diagnosed with JIA when she was ten years old. The condition subsequently affected multiple joints, leading to an arthroplasty of her right elbow and the replacement of both hips and knees. It was not clear from the notes what types of replacements these were. Her history included degenerative scoliosis. An accident in November, whilst trying to load her washing mashing using an adapted stick, led to a possibly fractured right elbow The lady had a family history of osteoarthritis affecting her brother and grandparents.

Investigations

X-ray: This showed that the right hip was chronically dislocated with the formation of a pseudoacetabulum.

MRI: This was performed to rule out the possibility of osteomyelitis as the pressure sores were necrotic and 1.5cm deep. The report noted a deep gluteal ulcer reaching distal to the coccygeal segment but no signs of osteomyelitis.

Bloods: Inflammatory markers showed raised CRP levels. Blood cultures were negative for MRSA as were wound swabs, but these were being repeated.

Differential Diagnosis

The MRI ruled out the differential diagnosis of osteomyelitis. There was the possibility that the patient had infected pressure sores as her CRP was raised. She has had bouts of diarrhea, vomiting and pyrexia, which could signify gastroenteritis.

Treatment

She was being given the antibiotics Flucloxacillin and Gentamicin for Gram positive and negative cover. Her pressure sores were being treated conservatively using Vacuum-assisted closure. It was not clear in her notes what medications she was taking for her arthritis, but she used to have monthly gold injections. She was being mobilized by the physiotherapists and was able to sit out in the chair on the day of my second visit, for the first time since her admission.

This patient was not ready to be discharged as her pressure sores and general health had deteriorated after I originally saw her. There was the possibility that her right hip would need replacing and her right elbow may also have a prosthesis fitted, once she had made an initial recovery. The patient mentioned that she would be moved to a rehabilitation centre closer to her home so that she could continue her recovery before returning home.

Discussion

What is juvenile idiopathic arthritis? JIA involves inflammation of joints which lasts for longer than six weeks. It is of unknown origin and occurs before the age of sixteen. It is the most common type of arthritis that presents during childhood. It causes systemic upset, synovitis affecting one or multiple joints, cartilage erosion and can lead to a number of extra-articular manifestations such as pericarditis. In around 30% of affected patients, arthritis can persist into adult life. JIA is an umbrella term which includes subtypes such as systemic arthritis, oligoarthritis and polyarthritis. Oligoarthritis is the most common subtype in developed countries and is more common in females under the age of six.

Diagnosis

The diagnostic criteria for this disease include daily fever of >39 degrees Celsius, arthralgia/ arthritis, Rhesus factor and antinuclear antibody (ANA) negative. There can also be a raised white cell count, rash, serositis, hepatosplenomegaly and adenopathy. The presence of Anti-cyclic citrullinated peptide (Anti-CCP) can be an indicator of severe disease. There are many possible differential diagnoses of JIA and it is important to rule out sinister diseases such as septic arthritis by performing a joint aspiration.

Management

Recent advancements in biologics mean that the treatment options for JIA are more successful at controlling the progression of the disease. The previous treatment consisting of NSAIDs and corticosteroids are now used alongside biologics and disease-modifying antirheumatic drugs (DMARDs) such as methotrexate. According to the British Medical Journal (BMJ) Best Practice, first line treatment of early disease should include changes to lifestyle, such as increasing exercise, and multidisciplinary input. For long term care, DMARDs such as Methotrexate or Sulfasalazine are indicated. Second line medications include the biologics Etanerept, Tocilizumab, Abatacept or Adalimumab for polyarticular JIA. Other options include Canakinumab, infliximab or Anakinra.

The National Institute for Health and Care Excellence (NICE) has guidelines for the treatment of JIA that encourage the use of biologics. These guidelines recommend that Tocilizumab be used in in children aged two years or older. Tocilizumab should be offered to a patient with systemic or polyarticular JIA that has not responded to NSAIDs, systemic corticosteroids or methotrexate. Abatacept is offered to people six year or older that have not responded to DMARDs or a Tumour necrosis factor (TNF) inhibitor. Adalimumab and Etanercept are given to children two years or older that haven’t responded to DMARDs like Methotrexate.

Complications

There have been extensive advances in medication options over the last twenty years. However, for people diagnosed prior to this, medication options were sparse and came with extensive side effects. The lady in this case report was diagnosed over fifty years ago, so did not benefit from the recent treatments to prevent her disease from progressing. The possible complications from JIA are varied according to the subtype of JIA. This case report will discuss some of the main problems mentioned in studies. If medications can’t control the synovitis, there can be joint destruction from resultant erosion of bones. Arthritis can cause a loss of function and possibly significant pain, it may even be necessary to undergo joint replacement. This can cause further longer term problems as the artificial joints may only last for 10-15 years, requiring re-replacement and in the process disrupt growth plates, leading to reduced long bone growth and leg length discrepancy.

Osteopenia and Osteoporosis can occur with certain subtypes of JIA. The effect is multifactorial and includes reduced mobility, muscle atrophy and pro-inflammatory cytokine release when the disease is active16. A study by Lien et al. demonstrated that 41% of patients with early onset disease developed low bone mass eleven years after diagnosis. This was related to the extent and duration of the disease. Compression fractures of the vertebrae are particularly apparent if the disease is very active, the patient has a high body mass index and had recently been using glucocorticoid treatment. However, this finding was not associated with having a low bone density. This is interesting as low weight is a risk factor for developing vertebral fractures in the general population. Cervical spine fusion or subluxation can occur if the inflammation and disease is uncontrolled.

The temporomandibular joint (TMJ) can become involved, causing the jaw to be undersized. This can cause pain and limited forward movement of the jaw when opening the mouth. If significant, surgical intervention may be necessary once the facial bones are fully developed. There are a number of studies indicating that TMJ arthritis is associated with JIA and involvement of this joint should be monitored using MRIs. Uveitis is a common extra articular manifestation and can cause a loss of vision. It is vital to monitor the person’s vision using a slit lamp as the onset of uveitis can be asymptomatic and idiopathic early on, but can lead to more sinister complications such as cataracts and glaucoma. Associations increasing the chance of having uveitis include developing arthritis at a young age, being female and being ANA positive.

Severe outcomes in JIA are reducing as a result of modified treatments. Previously, flexion contractures, weakness and poor ambulation were considered common complications of JIA before the introduction of biologic agents. These are now rare findings and so it will be interesting to determine the impact that the new treatments have on the long-term complications with future studies. There is concern that the use of anti-TNF treatment could be associated with an increased risk of malignancy. However, it has been hard to distinguish the exact cause for this possibility as it may result from the inflammatory disease process itself. Other trials suggest that the outcome of malignancy is similar to that experienced in the general population. Long-term follow up studies will need to be done in order to confirm this.

The morbidities associated with JIA can have a debilitating effect on patient’s general health and social lives. A reduction in mobility can lead to isolation and an impact on the prospect of having proper schooling or getting a job29. Sufferers become reliant on carers as it becomes harder to carry out the normal activities of daily living. This may become more apparent later on when the disease has had a greater impact and natural degeneration with age makes it harder to adapt to functional immobility. The lack of mobility in the patient I spoke to undoubtedly have a role in the development of her pressure sores as she was unable to relieve pressure on these areas. She will now need an elbow replacement as a result of a fracture sustained whilst attempting to do her own washing.

Learning Points

The debilitating effects of JIA, which are exemplified by this particular patient, emphasise the need for targeted and advanced treatment regimes. Fortunately, the recent development in medications has proven its efficacy by improving remission rates and reducing the patient’s burdens of morbidities. Alongside medical intervention, it is important to have a multi-disciplinary input to improve the holistic quality of care that this patient receives, especially when the patient is trying to cope at home. There is little information about how the burden of complications will change with long term use of these biologics. However, there is concern that there may be an increased risk of malignancy when using these new drugs and this is something being monitored in long term trials.

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