Rheumatic Diseases Primarily Affecting The Joints
Juvenile idiopathic arthritis/Juvenile Rheumatoid Arthritis
Both juvenile idiopathic as well as rheumatoid arthritis are terms used to describe the same disease. Traditionally the term rheumatoid arthritis has been used but internationally it has now been agreed to call the disease juvenile idiopathic arthritis. Arthritis is best described by four major changes in the joints that may develop. The most common features of JRA are: joint inflammation, joint contracture (stiff, bent joint), joint damage and/or alteration or change in growth. Other symptoms include joint stiffness following rest or decreased activity level (also referred to morning stiffness or gelling), and weakness in muscles and other soft tissues around involved joints.
This type of arthritis involves the spine as well as tendons, especially at the spots where the tendons attach to the bone (enthesis or enthesopathy).
Juvenile Psoriatic Arthritis
Psoriatic arthritis is an inflammatory joint disease associated with psoriasis, a chronic skin disease. Psoriasis is an inflammatory skin disease that results in itchy patches of skin that have a red base with a silvery scale on top. The rash most commonly appears on the elbows, knees and scalp, but may be found anywhere on the body. The fingernails and toenails also are commonly affected by psoriasis, with pitting, separation of the nail from the underlying nail bed, and transverse ridging and cracking. In a significant number of patients arthritis is associated with Psoriasis and will then be called Psoriatic Arthritis. It often presents with swollen fingers or toes. In affected digits, the whole finger or toe is usually swollen which is then called a “sausage digit”.
Following any type of infection, transient or chronic arthritis might occur. Children with chronic arthritis might be referred for assessment and a treatment plan.
2) Connective Tissue Diseases
Systemic Lupus Erythematosus
Systemic means affecting many organs or systems of the body. The word signifies that in this disease both internal and external parts of the body can be involved. Lupus means wolf. It refers to the rash on the face, which reminded doctors of the white markings present on a wolf's face. Erythematosus in Latin means red, and refers to the redness of the skin rash, which is very common in lupus.
Juvenile Dermatomyositis (JDMS)
JDMS is the most common form of myositis in children. It is a disease that causes a skin rash and weak muscles in children. The skin rash and weak muscles of JDMS are caused by inflammation in the blood vessels that lie under the skin and in the muscles. Since blood vessels run throughout the body, JDMS can also affect other areas such as the digestive tract. Usually, the worst symptoms are in the skin and muscles.
Scleroderma literally means “hard skin,” which is a finding common to a group of diseases that involves the abnormal growth of connective tissue. Scleroderma has come to be the term used for many of these related diseases. They fall into two main categories: localized scleroderma, which affects limited parts of the body, and systemic sclerosis, which can affect many areas of the body.
Raynaud’s phenomenon occurs when the blood vessels that feed your fingers and toes constrict in reaction to cold or emotional stress (this event is called a vasospastic attack).
Vasculitis means inflammation of blood vessels. In addition to Lupus erythematosus, Scleroderma, where vasculitis is a major component of the disease, a wide selection of other forms of vasculitis manifesting itself locally or systemically are being treated by pediatric rheumatologists.
4) Periodic Fever Syndromes
In recent years a number of diseases have been recognized that manifest itself by recurring fever episodes together with a varying array of other symptoms including joint pain/arthritis, skin rash and abdominal pain. Among them are the so called Familial Mediterranean fever, Hyperimmunoglobulinemia D with recurrent fever, TNF receptor associated periodic syndrome (TRAPS), Muckle-Wells syndrome, Familial cold urticaria, Neonatal onset multisystem inflammatory disease , Periodic fever - aphthous stomatitis - pharyngitis and adenitis (PFAPA syndrome, Blau syndrome, Pyogenic sterile arthritis - pyoderma gangrenosum - acne (PAPA syndrome)
5) Musculoskeletal Pain Syndromes
Sometimes children and adolescents are suffering from significant joint or muscle pain without any specific underlying disease. In these cases a diagnosis of musculoskeletal pain syndrome or pain amplification syndrome may be made after careful exclusion of other diseases. The treatment consists of a combined physiotherapeutic, psychological and medical approach.