Rheumatological disorders pose a challenge to clinicians because of
multisystemic involvement, relapsing-remitting course, and nonspecific clinical
features, which can mimic infections, malignancies, and even genetic disorders.
Common symptoms at presentation are joint pain, fever, weight loss, malaise, muscle
weakness, rash, and ulcers. While diseases, such as juvenile idiopathic arthritis,
juvenile dermatomyositis, and IgA vasculitis, are relatively easy to diagnose because of
typical clinical manifestations, others such as systemic lupus erythematosus,
scleroderma, and various vasculitides are much more challenging. No laboratory
investigation is diagnostic of a particular rheumatological disorder. Investigations, such
as antinuclear antibodies and antineutrophilic cytoplasmic antibodies, are associated
with a high false-positive rate and should be used judiciously. Most diseases except for
Kawasaki disease and IgA vasculitis are chronic and require long-term
immunosuppression for control of disease activity. Long-term prognosis has improved
over the past few decades due to better immunosuppressive regimens and better
monitoring. With an improvement in mortality rates, many children are living into
adulthood and facing issues with persistent disease activity and morbidity related to
therapeutic regimens. Future research should focus on finding better therapeutic
protocols, which should result in further improvements in survival while
simultaneously reducing drug toxicity. There is also an urgent need to define better
monitoring tools for most rheumatological conditions.
Keywords: ANCA associated vasculitis, Antiphospholipid syndrome, IgA vasculitis, Juvenile dermatomyositis, Juvenile idiopathic arthritis, Juvenile systemic sclerosis, Kawasaki disease, Localized scleroderma, Macrophage activation syndrome, Neonatal lupus, Polyarteritis nodosa, Rheumatological disorders, Systemic lupus erythematosus, Takayasu arteritis, Uveitis, Vasculitis.
