Page 842 - Clinical Immunology_ Principles and Practice ( PDFDrive )

814 Part SIX Systemic Immune Diseases

TABLE 59.1 Clinical Features of Giant-Cell Disability can be significant, as patients have difficulties with
arteritis (GCa), Polymyalgia rheumatica activities of daily living. With stenotic lesions of the subclavian
(PMr), and takayasu arteritis (ta) arteries, blood pressure readings can be unreliable or not possible,
requiring alternative strategies for blood pressure monitoring.
Organ FrEQUENCIES Although carotid involvement is considered infrequent, it can
System Clinical Features GCa PMr ta be challenging to distinguish atherosclerotic disease and vasculitic
disease. Patients with carotid GCA are at high risk for cerebral
Vascular Headaches +++ * ischemic events. Aortic involvement preferentially targets the
Limb claudication + *** thoracic aorta and infrequently the abdominal aorta (Fig. 59.6).
Scalp tenderness **
Jaw claudication ** Dilation of the aortic root can lead to aortic insufficiency. Aortic
Absent or asymmetrical * *** aneurysms are often clinically silent. The diagnosis may first be
pulses made from tissue obtained surgically during aortic aneurysm
Asymmetrical blood * *** repair. In extreme cases, the aortic wall ruptures.
pressure readings The response pattern of arteries to inflammation may not
Bruit * *** include intimal hyperplasia, thus eluding luminal compromise
Tongue claudication * and vascular failure. In such patients, the systemic inflammatory
Tissue gangrene *
Abdominal angina * component dominates the clinical presentation. Fever, fatigue,
Cough (dry, nonproductive) * * malaise, weight loss, and depression are often intense enough
Constitutional Malaise ** ** *** to prompt a workup for a malignancy. GCA needs to be a
Failure to thrive * ** * differential diagnosis in all cases of fever of unknown origin,
Weight loss ** ** ** particularly in older individuals. Patients with cranial GCA have
Fever * * * abnormally thick and tender temporal arteries exhibiting nodular-
Central Ocular symptoms ** * ity and loss of pulses, whereas clinical findings in nonstenosing
nervous
Stroke/transient ischemic * * GCA can be unremarkable. Temporal artery biopsy should
attack be pursued even if clinical examination does not suggest the
Peripheral Peripheral neuropathy * diagnosis.
nervous
Cardiac Aortic dilatation and * * CLINICAL FEATURES IN
regurgitation
Myocardial infarction * * POLYMYALGIA RHEUMATICA
Congestive heart failure *
Musculoskeletal Proximal stiffness/muscle ** *** PMR is diagnosed in patients presenting with pronounced
pain stiffness and pain in the shoulder and pelvic girdle muscles
2
Synovitis of peripheral joints * (see Table 59.1). Laboratory testing reveals a systemic inflam-
Others Intense acute-phase *** *** *** matory syndrome; arterial biopsy is negative for arteritis. It is
response estimated that about 10% of patients with PMR without any
Normochromic or ** * ** signs of vascular inflammation will eventually develop full-blown
hypochromic anemia
vasculitis. Notably, PMR often occurs in patients with GCA and
*** = high frequency (>70%); ** = moderate frequency (20–70%); * = low frequency is present in about 40% of patients with GCA at disease onset.
(<20%). Tapering of immunosuppressive therapy in GCA is frequently
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associated with new or remittent PMR symptoms. Complaints
are focused on muscle pain and stiffness, classically affecting the
loss is preventable, which indicates that GCA should be considered neck, shoulders, and pelvic girdle. The muscles of the torso may
an ophthalmological emergency. be involved. Peripheral areas of the arms and legs are spared.
Chronic nonproductive cough can be related to arteritis in Muscle pain is most intense in the early morning and improves
bronchial artery branches. If the vertebral and basilar arteries during the day. Inability to get out of bed, stand up from a chair,
develop vasculitic stenosis, ischemia of the central nervous system or get off the toilet seat should alert the physician to consider
manifests with transient ischemic attacks or frank stroke. PMR. Some patients with PMR have synovitis or bursitis in their
2,28
In patients with large-vessel GCA, cranial symptoms may be shoulder and hip joints, making it difficult to distinguish these
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minimal, and temporal artery biopsy can be negative. Instead, patients from those with seronegative polyarthritis. No diagnostic
vascular insufficiency is focused on the upper-extremity vessels procedure that allows for the diagnosis of PMR is available; the
and the aorta. In rare cases, lower extremities are affected. Typi- syndrome remains an exclusion diagnosis in cases of myalgia
cally, patients have asymmetrical blood pressure readings or combined with laboratory signs of systemic inflammation. On
experience total loss of upper-extremity blood pressure and pulse. clinical examination, passive motion of shoulder and hip joints
The underlying lesions are occlusions in the distal subclavian is seen to be maintained, but active motion is restricted because
arteries, often extending into the axillary sections (Fig. 59.5). of pain. Muscle strength is often normal. Careful evaluation
Patients with subclavian GCA are on average about 10 years of the temporal arteries is warranted to avoid missing fully
younger at disease onset compared with those with dominant developed GCA.
cranial manifestations. Diagnosis of large-vessel GCA can be
delayed as symptoms are nonspecific and the systemic inflam- CLINICAL FEATURES IN TAKAYASU ARTERITIS
matory component is less pronounced. Ischemic pain in the
hands when using the arms is often combined with coolness The clinical manifestations of TA are diverse and depend on the
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and bluish discoloration. Gangrene of the fingertips is rare. affected vascular territory (see Table 59.1; Table 59.2). Initial