Page 807 - Clinical Immunology_ Principles and Practice ( PDFDrive )

778 Part six Systemic Immune Diseases

involvement in AS is more common than once thought. The symptoms has been reported in approximately one-third of
clinical implications of these observations remain unclear as patients with AS, with women reporting more depression than
lung involvement in AS is usually asymptomatic. men. Pain was found to be a major determinant of depression
Renal manifestations. Renal involvement in AS, although for women but was of lesser importance for men.
uncommon, can include secondary renal amyloidosis (AA type), AS in women. AS in women may not be as severe as it is in
NSAID nephropathy, and glomerulonephritis. men and may present with isolated neck pain in the absence of
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Osteoporosis. Measuring bone mineral density in patients typical back pain. There tends to be a greater delay in the
with spondylitis is complicated by false increases in spinal density diagnosis of AS in women compared with that in men. Women
from dense syndesmophyte formation, leading some to recom- tend to have less severe involvement of the spine, with peripheral
mend quantitative CT over standard dual-energy X-ray absorp- joint involvement. A large review of the impact of AS on reproduc-
tiometry (DEXA) for bone mineral density measurements. tive events on women concluded that AS did not adversely affect
Nevertheless, up to half the patients with long-standing AS have the ability to conceive, pregnancy outcome, or neonatal health. 27
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been reported as having osteopenia or osteoporosis, which has
been attributed to the impact of inflammation on bone remodel- Reactive Arthritis
ing as a result of aberrant activation of bone morphogenic protein The classic triad of arthritis, urethritis, and conjunctivitis,
and Wnt signaling. This may be further worsened by treatment representing what was formerly known as Reiter syndrome, is a
factors and decreased mobility or physical activity, in addition presenting feature of only a minority of patients with ReA
to osteoclast/osteoblast imbalance. 25 (comprising only a third of the cases in some series). In ReA,
Spondylodiscitis and spinal fractures. An uncommon but the clinical features are nowadays viewed more as a spectrum
well-recognized complication of AS is spondylodiscitis, a destruc- ranging from the classic triad to undifferentiated SpA. In fact,
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tive discovertebral lesion also called Andersson lesion. Typically, the manifestations vary among patients, depending on the genetic
these lesions are confined to the thoracolumbar spine, sometimes makeup, the triggering event, and the sequential immunological
with multiple-level involvement. Pain and tenderness localized reaction.
to the affected disk are the most common presenting features Typically, the features start 1–4 weeks after a triggering event,
of spondylodiscitis, although it can be asymptomatic and only frequently identified as an enteric or urogenital infection, but
detected on routine radiographic examination many years later. often the event passes unnoticed without any specific symptoms.
Spondylodiscitis usually occurs at an advanced stage of AS under The syndrome starts with constitutional symptoms, such as
the form of an erosive condition related to both mechanical fatigue, malaise, and fever, and then is typically manifested by
factors and osteoporosis. However, early spondylodiscitis may asymmetrical, additive lower-extremity oligoarticular inflam-
occur as a result of the inflammatory process. Patients may or matory arthritis along with an array of different extraarticular
may not have a history of preceding trauma. features, including a sterile oligoarticular or monoarticular and
Even trivial falls can be catastrophic for AS patients, who are asymmetrical arthritis of the lower extremities, especially knees,
at risk for spinal fractures because of their spinal rigidity and ankles, and, occasionally, hips. Upper-extremity involvement is
osteoporosis. The estimated prevalence of vertebral fractures in encountered less commonly. Dactylitis occurs in the toes or
AS varies from 4% to 18%. Fractures through the disk space, fingers, resulting in the “sausage digits,” which represent inflam-
the weakest point in the ankylosed spine, are most common, mation not only of the interphalangeal joints of the hands and
with the cervical spine being the most frequently affected region, feet but also of the surrounding soft tissue structures, including
followed by the thoracolumbar junction, and may or may not tendons and subcutaneous tissue.
be complicated by injury to the spinal cord, ranging from mild Sacroiliitis and spondylitis are less common than peripheral
sensory loss to quadriplegia. Spontaneous atlantoaxial subluxation arthritis, although inflammatory back pain does occur. Unilateral
is also rarely seen. and bilateral sacroiliac involvement and even spondylitis occur,
Neurological manifestations. Neurological involvement in AS especially in those with chronic or long-standing disease. The
is most often related to spinal fracture, atlantoaxial subluxation, most common sites for enthesitis are the Achilles tendon and
or cauda equina syndrome. The cauda equina syndrome in AS plantar fascia insertions, although tenderness over the symphysis
is characterized by a slow insidious progression and a high pubis, iliac crest, ischial tuberosity, greater trochanters, and
incidence of dural ectasia, although a rapid onset secondary to thoracic cage ribs may also occur.
a traumatic event has been reported. It tends to be a late manifesta- Mucocutaneous lesions may be difficult to distinguish from
tion of AS, often when the disease is no longer active. The PsA, especially circinate balanitis and keratoderma blennorrhagica.
prevalence of neurological findings in cauda equina syndrome Circinate balanitis is an ulcerative mucosal lesion over the glans
in AS is very high, presenting with a prodrome of sensory, motor, or shaft of the penis that is demarcated by a serpiginous
or reflex loss before the progression to sphincter disturbance. erythematous border. The lesion is usually painless and sterile
About half the patients have pain in the rectum or lower limbs unless a superimposed infection occurs. Keratoderma blennor-
that is presumably neurogenic in origin. Case reports have also rhagica is a painless desquamative psoriatic-like papulosquamous
been published about the occurrence of AS with a multiple eruption and is sometimes referred to as pustulosis palmoplantaris
sclerosis (MS)–like syndrome and transverse myelitis, although and occurs on the palms and soles of the feet. Oral lesions have
the association is not conclusive. been described as shallow, painless ulcers or patches on the palate
Fatigue and psychosocial manifestations. Fatigue is a common and tongue, or mucositis of the soft palate and uvula. Conjunc-
problem in AS and seems to be associated with more severe tivitis and AAU also occur, as described in AS. Conjunctivitis
disease. Sleep disturbance has been reported to be as high as may be unilateral or bilateral and is usually an early feature
nearly 81% of female patients with AS and 50% of male patients. manifesting with irritation, erythema, and lacrimation. It is
The disturbance is closely related to pain during the night usually associated with a sterile discharge unless a superimposed
characteristic of active disease. A high level of depressive infection occurs because of eye rubbing. It can be severe and