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Diffuse idiopathic skeletal hyperostosis.

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DIFFUSE IDIOPATHIC SKELETAL HYPEROSTOSIS
164
not receiving x-ray therapy was studied (S258). The
number of cancer deaths in patients not treated with xray was not greater than that expected from national
death rates. Specifically, there were no deaths from leukemia. In spondylitis patients treated with x-ray, deaths
from leukemia and other forms of cancer were significantly increased above those not treated with x-ray. It
was concluded that the excess of leukemia and cancer is
a direct result of x-ray therapy and unrelated to the disease process itself.
DIFFUSE IDIOPATHIC SKELETAL HYPEROSTOSIS
Diffuse idiopathic skeletal hyperostosis (DISH)
is a recently proposed name for a skeletal disorder producing characteristic alterations in both spinal and extraspinal structures (R62). Although the name is new,
the disorder is not, having first been described over 35
years ago.
A report appeared that summarized the historical, clinical, radiographic, and pathologic findings of
DISH, based on a literature review and observations of
a large number of patients (R62). Previous designations
for DISH have included spondylitis ossificans ligamentosa, spondylosis hyperostotica, and (senile) ankylosing hyperostosis of the spine. While the latter has
been the most popular term, vertebral ankylosis may
not be found on pathologic examination, although it
may be apparent radiographically. Furthermore, extraspinal manifestations are common and may be more extensive than spinal alterations. In fact, extraspinal abnormalities may exist without appreciable vertebral
abnormalities. Consequently, the term DISH was introduced in order to emphasize the widespread nature
of this disorder.
Etiology and pathogenesis
The etiology of DISH is unknown (R62). It has
been proposed that DISH may not represent a true disease entity but rather a vulnerable state in which extensive ossification results from an exaggerated response of
the body in certain patients to stimuli that produce only
modest new bone formation in others. As such, DISH
would represent an ossification diathesis that causes excessive bone formation at skeletal sites subject to normal or abnormal stress. These sites are generally those
where tendons and ligaments attach to bone, both in the
axial and extraaxial skeleton. Additional evidence for
an ossifying diathesis includes the propensity of patients
to develop ossification in response to surgery or to coexisting diseases such as rheumatoid arthritis (RA) (R53).
HLA typing was performed in 47 white patients
with DISH (R62); 16 (34%) had the B27 antigen, suggesting that B27-positive subjects may be at risk for developing DISH. Moreover, because DISH and other
HLA-B27-associated arthropathies manifest abundant
new bone formation, the authors suggested an association between this antigen and the genes controlling new
bone formation.
However, in 3 subsequent reports of HLA typing
in DISH, with 50 patients in each, the frequency of B27
ranged from 4 to 8% (B241a,E61,R138). Conversely,
other HLA associations were disclosed in these 3 reports. The frequency of B5 was found to be twice that of
controls in one (E61), and 3 times that of controls in another (R138), while in the third report a significant decrease of the A9 and A l l specificities was disclosed
(B241a). [While the weight of evidence is against an association between DISH and the B27 antigen, these
conflicting data support the need for further study. Ed.]
DISH is a common disorder among Pima Indians, with prevalence rates approximating 50% in men
aged 55 and older (S300). Because of the increased
prevalence of both DISH and the HLA-B27 in Pima Indians, a possible association between the 2 was believed
to exist. However, when HLA testing was performed in
44 adult male Pima Indians age 55 or over with DISH
and in 33 age-matched controls, no association was
found. Specifically, the B27 antigen was found with
similar frequency in both patients (16%) and controls
(20%).
Clinical features
In order to diagnose spine involvement due to
DISH, 3 radiographic criteria were proposed (R62): 1)
the presence of flowing calcification and ossification
along the anterolateral aspect of at least 4 contiguous
vertebral bodies, 2) absence of extensive degenerative
disc disease, and 3) absence of both apophyseal ankylosis and sacroiliac erosion, sclerosis, or fusion. The first
criterion helps to separate DISH from typical spondy-
REITER’S SYNDROME
165
losis deformans, the second to distinguish it from intervertebral (osteo) chondrosis, and the third to eliminate
patients with ankylosing spondylitis. Although the spinal alterations in DISH are generally easy to recognize
on x-ray, this disorder must be distinguished from other
conditions of the vertebral column associated with hyperostosis (R52,62).
The principal musculoskeletal complaints reported by patients were spinal stiffness and mild mid- to
low-back pain (R62). Occasionally, cervical spine pain
was an initial and eventually prominent complaint.
Dysphagia may be an additional symptom, directly related to prominent cervical osteophytes. There appeared
to be few changes on physical examination of the spine,
except for a slight decrease of lumbar lordosis, minimal
increase of dorsal kyphosis, or minor distortion of spinal
mobility. Rarely were scoliosis, severe spinal rigidity, or
restricted thoracic cage motion noted. Extraspinal complaints were frequent, particularly recurrent tendinitis
or spurs of elbows and heels. Discomfort of other peripheral sites, such as shoulders, hips, knees, and ankles
was less frequent. Patients with extraspinal problems often had significant local osseous proliferation or spurs
when examined radiographically. There were no appreciable laboratory abnormalities, except for a low frequency of minimal elevation of both the erythrocyte
sedimentation rate and serum glucose level.
A report of 4 patients with DISH revealed exten-
sive calcification and ossification of the posterior longitudinal ligament of the cervical spine (R55). A subsequent
review of cervical spine x-rays in 74 additional patients
with DISH revealed bony hyperostosis of the posterior
aspect of the vertebrae in 41%, posterior spinal osteophytosis in 35%, and posterior longitudinal ligamentous
calcification and ossification in 50%. These findings,
previously described almost exclusively in Japanese patients, appear to be an additional skeletal manifestation
of DISH. Although patients with posterior longitudinal
ligamentous ossification may be asymptomatic, a variety of signs and symptoms may occur. Parathesias may
vary from intermittent sensations, including numbness
or tingling of several digits of one or both extremities, to
extensive and severe anesthesia of the trunk and lower
extremities. Motor disturbances such as weakness, incoordination, and instability may be encountered in upper and lower extremities. Additional symptoms may
include head and neck pain and stiffness, urinary and
rectal incontinence, and loss of libido. On physical examination, patients may have muscle atrophy, fasciculations, hyperreflexia, and sensory loss.
Eight patients with both DISH and RA were described (R53). An admixture of x-ray features, some
typical for RA and others for DISH, were observed.
These included absence of osteoporosis, presence of
bone eburnation and proliferation around erosions,
osteophytosis, and bony ankylosis of joints.
REITER’S SYNDROME
A comprehensive and provocative review of Reiter’s syndrome (RS) appeared (S 160). Evidence showing
a high correlation between RS, other seronegative arthropathies which share Reiter’s symptomatology, and
the histocompatibility antigen HLA-B27 was reviewed.
It was suggested that HLA-B27 can be used as a confirmatory test in diagnosis. It was also suggested that the
correlation of HLA-B27 with similar arthropathies indicates that these conditions are not separate entities
but are more than likely different manifestations of the
same disease process. It was further urged that the name
“Reiter’s disease” be abandoned and a new designation
which more accurately describes the spectrum of this
disorder be adopted.
Etiology and epidemiology
HLA-B27 was detected in 80% of 173 patients
studies (K150). An increase in the frequency of HLAA2, in proportion to that of B27, was also noted. In another series, B27 was present in 34 patients (78%) and
absent in 10 (A139). Of the 10 B27-negative patients, 7
had antigens of the B27-cross-reactive group (Creg), including B7 in 2, Qw22 in 4, and Bw42 in 1. Mechanisms
to explain the association of HLA-B27 with RS and ankylosing spondylitis, including abnormal immune response genes linked to HLA or a direct role of HLA antigens in disease pathogenesis, were investigated (A 139).
Data supporting the latter hypothesis were provided.
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diffuse, skeletal, idiopathic, hyperostoses
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