‘Timely treatment can limit disability in children with Juvenile Arthritis’
Another World Young Rheumatic Disease Day (WORD Day) went by (on March 18) with experts drawing attention to raising awareness and knowledge to help in the early diagnosis of rheumatic diseases among young people. Lack of awareness and delayed diagnosis remains a cause of concern for specialists.
Narendra Bagri, additional professor, Division of Paediatric Rheumatology, Department of Paediatrics, All India Institute of Medical Sciences, New Delhi laid emphasis on prompt diagnosis and timely treatment of Juvenile Idiopathic Arthritis (JIA) to prevent complications as well as the need to increase awareness in the community.
JIA, he said, is a broad spectrum of inflammatory arthritis encompassing various subtypes and is the most common paediatric rheumatic disorder. “The worldwide prevalence of JIA is variable, ranging from 0.07 to four per 1,000 children. There is a global variation in the distribution of various subtypes; for instance, oligoarticular JIA ( fewer than four joints are involved) is more common in the western world, while enthesitisrelated arthritis characterised by pain over heels and lower back pain is the common subtype in the Indian subcontinent accounting for nearly onethird or more of cases,” he said.
Though there is a lack of a national registry, given the population of the country, a sizeable number of children with arthritis suffer from JIA, he said.
Symptoms, treatments
Children often present with joint pain and swelling with functional limitations such as limping, he said, adding: “the symptoms are more pronounced in the morning when they get up from bed or after a long period of rest. This phenomenon of morning stiffness may be reported by older children, while smaller kids may find it difficult to express. However, parents must look out for poor activity levels early in the morning, which may improve as the day passes.”
Depending on the subtype of JIA, the number of joints may vary from a few to many, and accordingly, the resultant functional limitations would also vary. For example, involvement of joints of the lower limbs (knee, ankle) and lower back (sacroiliitis) may impair walking, while wrist joint and upper limb involvement would affect writing. The temporomandibular joint ( jaw joint) can lead to a difficulty in opening the mouth and eating. Delayed diagnosis and uncontrolled arthritis can leave children crippled and bedridden, Dr. Bagri said.
Apart from arthritis, these children can have other symptoms such as pain where the ligament or tendon originate (known as enthesitis). This may manifest as heel pain. Fever, rash and redness of the eyes may be other accompanying features, depending on the subtype of JIA. Young children with JIA can also develop asymptomatic inflammation in part of the eye (uveitis), which may be visionthreatening, and these children should also be proactively screened for this complication. In addition to JIA, arthritis can also be a manifestation of other paediatric rheumatic disorders, such as childhood lupus and juvenile dermatomyositis, which might be identified by a peculiar skin rash.
Numerous drugs are now available for the management of JIA. “Unlike in the past, the current era has challenged the therapeutic nihilism clouding the treatment of these disorders. Today, there are many effective drugs for the treatment of JIA. Broadly, the drugs used are steroids (intraarticular injected locally in the joint or in some cases, prescribed systemically), diseasemodifying antirheumatic drugs (DMARDS) or newer drugs such as biologicals. As these drugs modulate the heightened immune system that causes arthritis, and may have side effects, they must be used under the close supervision of a physician, and selfprescription is a strict no,” Dr. Bagri said.
Effective drugs
There are effective drugs, including biologics/biosimilars in addition to the conventional DMARDS which are not only effective but also minimise the side effects of systemic steroids used for some of these children. “Although effective, they are still out of reach for the common man and hence, there is a need for a collective effort to ensure their availability,” he said.
Studies demonstrate that there is a window of opportunity early in the course of the disease during which appropriate management improves outcomes.
A paper has been written on factors that predict a visit to a paediatric rheumatologist within 3 months, or periodic reviews. It is titled: ‘Factors impacting referral of JIA patients to a tertiary level pediatric rheumatology centre in North India: a retrospective cohort study in Paediatric Rheumatology by Manjari Agarwal et al’. It found these factors to include: prosimity to the doctor, family history of inflammatory disease, history of fever, history of acute uveitis (inflammation in the eye) or a high ESR, indicating inflammation in the body. The authors averred that cost of care and a remote treatment centre delayed consultation; acuity of complaints and family history of rheumatologic condition hastened referrals. Among their solutions would be to increase the number of centres with paediatric rheumatologists and to improve medical insurance coverage
In addition to drugs, physiotherapy is also an integral pillar for the management of JIA.
“With effective and timely treatment, the burden of disability in these children can be curtailed to a minimum and a functionally independent adulthood can be ensured.”
However, there are a number of challenges; one of the most important being the lack of awareness about these disorders in the community.
“People often are not aware of the fact that rheumatic disorders can affect kids too. Another alarming concern is delayed diagnosis as many of these children may display slow development of the disease, which may delay seeking of appropriate medical advice. Additionally, they may also be misdiagnosed under the rubric of infectious arthritis. Infections are common in our settings, but the pattern of arthritis in children with JIA is usually different from infectious arthritis, such as tubercular arthritis (not every chronic arthritis is tubercular). So, it is pivotal to ascertain the cause of arthritis before initiating treatment,” he elaborated.
Better awareness of JIA and its symptoms among primary care providers could help in streamlining the referral pattern of children. They should be managed by a team comprising paediatric rheumatologists, physiotherapists, ophthalmologists and other physicians.
The consequence of an untreated and prolonged illness would be a permanent disability, the extent of which would depend on the subtype of JIA.
Apart from limbthreatening complications, systemic JIA can have serious lifethreatening complications as well, he said.
(serena.m@thehindu.co.in)
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