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.
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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 enthesitis-related arthritis characterised by pain over the heels and lower back pain is the common subtype in Indian subcontinent accounting for nearly one-third or more of cases,” he said.
Though there is no national registry, given the population of the country, a sizeable number of children with arthritis suffer from JIA, he said.
There is a window of opportunity early in the course of the disease, during which appropriate management improves outcomes. | Photo Credit: Getty Images
Symptoms and 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 resting a while. This phenomenon of morning stiffness may be reported by older children, while smaller kids may find it difficult to express. However, parents can look out for limited activity 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 limb (knee, ankle) and low back (sacroiliitis) may impair walking, while wrist joint and upper limb involvement affect writing. The temporomandibular joint (jaw joint) can create 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 vision-threatening, 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. Many effective drugs can be used for the treatment of JIA. Broadly, the drugs used are steroids (intra-articular - injected locally in the joint or in some cases, prescribed systemically), disease-modifying 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 self-prescription is a strict no,” Dr. Bagri said.
Effective drugs now available
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 children. “Although effective, they are still out of reach of the common man and 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 for 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 inPaediatric Rheumatology’ by Manjari Agarwal et al.
It found these factors to include: proximity 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 the cost of care and a remote care facility 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 of JIA management. “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 are often unaware of the fact that rheumatic disorders can affect kids too. Another alarming concern is delayed diagnosis as many of these children may have a slowly developing disease, which may delay seeking 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 untreated and prolonged illness would be a permanent disability, the extent of which would depend on the subtype of JIA. Apart from limb-threatening complications, systemic JIA can have serious life-threatening complications as well, , he said.
