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Arthritis can be a significant
feature of each of the diseases discussed in this chapter. Description
of the disorder is brief and limited to the rheumatic manifestations
of the disease. More detailed discussion of each entity is found in
other chapters devoted to these diseases.
HYPERLIPOPROTEINEMIA
In familial hypercholesterolemia (type II hyperlipoproteinemia) recurrent
episodes of acute migratory polyarthritis occur in homozygous cases.
In heterozygous patients Achilles tendinitis, monoarthritis, or polyarthritis
of brief duration and variable severity can involve both large and small
joints. Tendinous xanthomas appear in late stages of this disease. In
some individuals with hypertriglyceridemia (type IV hyperlipoproteinemia)
a mild asymmetric osteoarthritis of chronic or recurrent type has been
observed. Several of these patients have had periarticular bone cysts
identified in joint radiographs. Certain of the reported cases may have
occurred in association with familial combined hyperlipidemias which
includes individuals with both of these plasma lipoprotein profiles.
Several authors have reported gradual reduction in the severity and
frequency of articular symptoms after correction of the plasma lipid
abnormalities by appropriate dietary and drug therapy.
SARCOIDOSIS
The most common rheumatic manifestation of sarcoidosis is an acute,
symmetric polyarthritis associated with erythema nodosum and hilar adenopathy
(Löfgren's syndrome). The ankles are most frequently involved, followed
by the wrists, the proximal interphalangeal joints, and the elbows.
Arthritis and the other acute manifestations usually resolve sponta-
neously within a few weeks without sequelae. Circulating immune complexes
and an increased frequency of HLA-B8 are found in these patients. Treatment
with salicylates or corticosteroids has been symptomatically beneficial
in individual cases. Chronic granulomatous sarcoid synovitis is an uncommon
form of osteoarthritis that can cause joint destruction. It is less
responsive to drug treatment and follows a variable clinical course.
HEMOCHROMATOSIS
Joint involvement has been observed in approximately one half of patients
with idiopathic hemochromatosis. Joint swelling with bony enlargement
is particularly common in the small joints of the hands, but the wrists,
hips, and knees may also be affected. The clinical and roentgenographic
features resemble osteoarthritis more than inflammatory joint disease.
There is roentgenographic evidence of narrowing of the joint space with
subchondral erosions and sclerosis. Chondrocalcinosis is present in
about 50 per cent of patients with arthropathy and may lead to acute
episodes of crystal-induced synovitis. Management of this arthropathy
includes the use of a nonsteroidal anti-inflammatory drug and prosthetic
weight-bearing joint replacements in advanced disease.
SICKLE
CELL DISEASE AND OTHER HEMOGLOBINOPATHIES
Severe polyarthralgia is a frequent manifestation of the crises of sickle
cell disease. Occasionally the pain is accompanied by transient joint
effusions. Skeletal abnormalities result from widening of bone marrow
spaces and focal sickle cell thrombosis in bone. The most common bony
lesion is avascular osteonecrosis of the femoral head; less commonly
the humerus and vertebral bodies are involved. This compli- cation is
also associated with sickle cell trait, hemoglobin C disease, sickle
cell disease, and sickle cell-thalassemia disease. In children, peritonitis
may result in transient diffuse swelling of the hands and feet (dactylitis).
Sickle cell disease is associated with an increased incidence of bacterial
arthritis and osteomyelitis, especially those caused by gram-negative
organisms. Arthropathy in patients with the thalassemia syndromes.
FAMILIAL MEDITERRANEAN
FEVER
Joint involvement is second only to peritonitis as the most common manifestation
in this disease. The arthritis is usually monoarticular and acute in
onset and most commonly affects the large weight-bearing joints. The
articular attacks remit in a few days, and only in a few cases do joint
symptoms persist for weeks to months. Arthritis, like other manifestations
of this syndrome, is recurrent, but permanent damage to joints other
than the hip is rare.
HYPOGAMMAGLOBULINEMIA
A polyarthritis, rarely deforming in character, has been observed in
as many as one third of patients with congenital or acquired hypogammaglobulinemia.
The pattern of joint involvement resembles that of rheumatoid arthritis;
in addi- tion, other connective tissue diseases such as systemic lupus
erythematosus, systemic sclerosis, and dermatomyositis have been associated
with immune deficiency states. A variety of autoimmune phenomena and
connective tissue syndromes, including juvenile arthritis, have been
observed in patients with selective IgA deficiency. Regression of arthritis
has been observed following institution of gamma globulin therapy
HYPERPARATHYROIDISM
Patients with hyperparathyroidism are subject to a variety of associated
rheumatic disorders that may occur singly or in combination. These include
hyperglycemia and gouty arthritis, chondrocalcinosis with episodes of
calcium pyrophosphate dehydrate crystal-induced synovitis (CPPD disease),
and osteoarthritis resulting from deformation of atrophic subchondral
bone. Rheumatic symptoms, particularly those associated with CPPD disease,
may be the first manifestations of hyperparathyroidism.
ACROMEGALY
The majority of patients with acromegaly develop an atypical form of
osteoarthritis. Increased levels of growth hormone result in hypertrophy
of articular cartilage, subchondral bone, and periarticular tissues.
Hypermobility of joints, a common manifestation, may contribute to degenerative
change. The fingers and knees are most frequently affected. Pathognomonic
radiographic features include overgrowth of bone and cartilage. Median
nerve entrapment (carpal tunnel syndrome) secondary to wrist synovitis
can occur.
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