Less-common arthropathies Medical Assignment Help

Amyloidosis

Primary amyloidosis causes a polyarthritis that resembles RA in distribution and it is also often associated with carpal tunnel syndrome and subcutaneous nodules. Ankylosing vertebral hyperostosis (Forrestier’s disease)
This is a condition of elderly people in which exuberant osteophytes are found in the spine, particularly the dorsal region. It is often asymptomatic, but it may be confused with ankylosing spondylitis. Sometimes there is stiffness and occasionally some discomfort. It may be accompanied by peripheral soft-tissue problems associated with calcification, ossification and spur formation, when it is called diffuse idiopathic skeletal hyperostosis (DISH).

Avascular necrosis

In this condition, bone infarction disrupts the surface of the joint and often leads to changes resembling OA. The hip is particularly affected, for example following fractures of the neck of the femur, perhaps because of its precarious blood supply. It also occurs in various systemic conditions, including sickle cell disease, prolonged steroid therapy, SLE, alcohol abuse and Gaucher’s disease. It occurs in deep-sea divers (caisson disease). It may occur for no obvious reason, particularly in middle-aged men. Avascular necrosis presents with a single painful joint. X-rays are usually diagnostic in the latter phases, showing rarefaction and dense bone in the subcortical areas with distortion of the epiphysis. In the early stages, a bone scan will show increased uptake and an MRI scan will confirm the diagnosis.

Behcet’s syndrome

This is a rare condition characterized by oral and genital ulceration, iritis, and a polyarthritis of variable distribution that may be either chronic or episodic. There are many less-common features of the disease, including erythema nodosum, pustular skin lesions, and neurological and gastrointestinal manifestations. Treatment is with oral steroids but response is variable.

Drugs

Drugs may cause arthritis. SLE may be induced by procainamide, hydralazine and other drugs. Certain drugs may also precipitate or aggravate gout.

Familial Mediterranean fever

This condition occurs in certain ethnic groups, particularly Jews and Arabs. The aetiology is unknown. It is characterized by recurrent attacks of fever, arthritis and abdominal or chest pain due to pleurisy. The arthritis is usually monoarticular and attacks last up to 1 week. The condition may be mistaken for palindromic rheumatism, but such attacks are not usually accompanied by fever. In familial Mediterranean fever, attacks can usually be prerented by regular treatment with colchicine 1.0-1.5 mg daily. In general the disorder is benign but in some cases amyloidosis develops.
Hypermobility syndrome Hypermobility syndrome occurs in children or young adults with lax joints. The musculoskeletal manifestations f this syndrome include recurrent attacks of joint pain and effusion, dislocation, ligamentous injuries, low back oain and premature OA. Hypermobility is also associated ith some rare congenital disorders such as the EhlersDanlos syndrome. Joints become stiffer with increasing age. Treatment should be directed at improving muscle power.

Osteochondritis dissecans

This is a rare condition of adolescent sportsmen in which there is separation of an avascular osteochondral fragment in the knee joint. It results in disruption of the joint surface and the formation of loose bodies within the joint. OA may develop.

Osteochondromatosis

In this condition, foci of cartilage form within the synovial membrane. These foci become calcified and then ossified (osteochondromas). They may give rise to loose bodies within the joint. The condition occurs in a single joint of a young adult and X-rays are usually diagnostic. Treatment involves removal of loose bodies and synovectomy.

Palindromic rheumatism

This condition is a variant of RA, characterized by recurrent attacks of arthritis. They occur at irregular intervals, sometimes with long periods of freedom between them, begin suddenly, reach a peak after a few hours and fade over the course of the next 2 days. A single joint is affected in each attack and during the attack it is red, warm, swollen and very painful. Between attacks the joints are normal. Tests for rheumatoid factor are positive in 50% and one-third of cases go on to develop chronic RA. Pigmented villonodular synovitis This is characterized by exuberant synovial proliferation that occurs either in joints or in tendon sheaths. The main manifestation in joints is recurrent haemarthrosis. Treatment is synovectomy. In tendons, the condition gives rise to a nodular mass that requires excision.

Relapsing polychondritis

Relapsing polychondritis is a rare condition of cartilage. It gives rise to a polyarthritis accompanied by chest disease due to tracheal or bronchial involvement. The diagnosis is often made on the basis of recurrent attacks of pain and swelling of the nose or external ear.

Temporomandibular

pain/dysfunction syndrome

This is a functional disorder of the temporomandibular joint associated with abnormalities of bite. It particularly occurs in anxious people who grind their teeth at night.It gives rise to pain and clicking in one or both temporomandibular  joints. Treatment is dental-correction of the bite.

Sarcoidosis

The commonest type of arthritis is that associated with erythema nodosum, which occurs in 20% of cases of sarcoidosis at or soon after the onset of the disease. The most useful diagnostic test is a chest X-ray, which shows hilar lymphadenopathy in 80% of cases. Other patterns of arthritis including a transient rheumatoid- like polyarthritis and an acute monoarthritis that can be mistaken for gout occur later in the course of the disease. If NSAIDs fail to control the symptoms, corticosteroids are usually very effective.

Posted by: brianna

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