Pulmonary Manifestations of Ankylosing Spondylitis
Section snippets
Demographic features
Ankylosing spondylitis is estimated to affect 0.1% of the general population.8 However, there is significant variability in prevalence, ranging from approximately 2 per 1000 individuals among black South Africans to 63 per 1000 individuals among Canadian Bella Bella Indians.9 A strong familial pattern has been described with an estimated 10% increased likelihood of the disease identified among first-degree relatives of an affected individual.3
The disorder affects men more commonly than women.
Pathophysiology
Ankylosing spondylitis has been described in persons with amyloidosis, psoriasis, regional enteritis, systemic sclerosis, ulcerative colitis, and urogenital infections. These associations suggest that an inflammatory or immunologic process is responsible for its pathogenesis.2 It has been proposed that cross reactivity between microbial elements and specific histocompatibility antigens may trigger an altered immune reaction. Klebsiella-related antigens have been identified in persons with
Clinical features
Inflammation of the sacroiliac joints is an early manifestation, and patients may present with back pain or morning stiffness. Other joints of the axial skeleton and extremities may eventually be affected also. About 30% of the patients have peripheral joint involvement.3 Pain on inspiration secondary to limited chest wall expansion and straightening of the lumbar spine can occur.2
Ankylosing spondylitis can affect the ocular, cardiovascular, renal, and neurologic systems (Box 1).
Respiratory manifestations
The presence of pleuropulmonary disease in patients with ankylosing spondylitis was first described by Dunham and Kautz15 in 1941 and by Hamilton16 in 1949. Pulmonary involvement in ankylosing spondylitis consists most frequently of abnormalities of the thoracic cage and lung parenchyma.5, 6 In one study, pleuropulmonary involvement was observed in 28 (1.3%) of 2080 patients with ankylosing spondylitis,17 including 25 cases of apical fibrobullous changes, 2 of pleural effusions, and 1 of apical
Clinical course
Onset of symptoms is usually insidious and generally starts between 19 and 40 years of age.2 The disorder is characterized by progressive loss of functional capacity, with most of the impairment occurring in the first decade of the disease.42 Gran and Skomsvoll42 conducted a long-term study of 100 patients with this disorder, more than half of whom remained employed in full-time work after a mean disease duration of 16 years. The presence of “bamboo spine,” acute anterior uveitis, and
Evaluation
Many inflammatory and connective tissue diseases share features in common with ankylosing spondylitis, and laboratory tests may be required to help distinguish them. Patients with ankylosing spondylitis may demonstrate B-lymphocyte reactivity, circulating immune complexes, elevated levels of immunoglobulins, and increased serum levels of alkaline phosphatase and creatine phosphokinase.45 Bronchoalveolar lavage may be normal and may show no evidence of alveolitis or may reveal increased levels
Therapy of ankylosing spondylitis
No treatment has been shown to alter the clinical course of apical fibrobullous disease.1, 2, 3, 49 Therapy with antiinflammatory agents, such as phenylbutazone or diflunisal, for arthritic symptoms does not improve pulmonary function or halt the progression of the disease. Management of respiratory complications of ankylosing spondylitis is mainly related to treating pulmonary superinfections using antifungal or antibacterial agents, which are either administered systemically or instilled
Summary
Several pulmonary disorders have been described in patients with ankylosing spondylitis, and these can be associated with significant morbidity and mortality. Management is mainly aimed at treating pulmonary superinfections. New antiinflammatory agents for arthritic symptoms seem to have no significant effect on the natural course of pulmonary disorders in these patients.
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