(Chest. 2000;118:1814-1816.)
© 2000
American College of Chest Physicians
Upper Lobe Fibrocavitary Disease in a Patient With Back Pain and Stiffness*
Divya Thai, MD;
Rita S. Ratani, MD;
S. Salama, MD, MPH and
Robert M. Steiner, MD, FCCP
*
From the Departments of Pulmonary Medicine and Radiology, New York Methodist Hospital and Weill Medical College of Cornell University, New York, NY.
Correspondence to: Rita Ratani, MD, Department of Radiology, New York Methodist Hospital, 506 Sixth St, Brooklyn, NY 11215; e-mail: rratani{at}hotmail.com
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Introduction
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A 37
-year-old white man presented with a productive cough, anorexia, weight
loss, and night sweats of 4 months’ duration. He also complained of
back pain with stiffness. The back pain was worse at night. He was
treated for pneumonia 3 years ago and had a 20 pack-year history of
smoking.
He was a thin man in no distress. His vital signs were stable and
temperature was 38.6°C (101.6°F). Bilateral enlarged firm
but nontender axillary and inguinal lymph nodes were noted. Chest wall
expansion was limited, but otherwise the thoracic examination was
unremarkable. There was kyphosis of the lumbar spine with limited
forward bending, The range of motion of the cervical spine was severely
limited, and there was no lateral motion of the neck. The pelvis was
anteverted, and there was a flexion deformity of both knees.
Laboratory findings were as follows: WBC count, 11,300
cells/µL; and erythrocyte sedimentation rate, 30 mm/h. There
was a positive human leukocyte antigen-B27 antigen. Sputum smears and
cultures for acid-fast bacilli were negative. Smears of BAL fluid and
bronchial washings revealed rare acid-fast bacilli. The purified
protein derivative skin test was negative.
A chest radiograph (Fig 1
) revealed bilateral upper lobe cavitary lesions. The remainder of the
lungs were hyperinflated. There was mild upward retraction of the hilar
structures indicating upper lobe volume loss. Contrast-enhanced chest
CT (Fig 2
) showed distortion of the lung architecture with bilateral upper lobe
cavitation and bronchial thickening. A radiograph of the cervical
spine (Fig 3
) demonstrated straightening of the cervical spine with squaring of the
vertebral bodies and calcification of the anterior longitudinal
ligaments. There was ankylosis of both sacroiliac joints (Fig 4
).
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Figure 1.. Posteroanterior chest radiograph reveals bilateral
upper lobe cavitary lesions. The lungs are hyperinflated. Mild upward
retraction of the hilar structures suggests upper lobe volume loss
related to fibrosis.
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Figure 3.. Oblique radiograph of the cervical spine
demonstrates straightening of the cervical spine with squaring of the
vertebral bodies and calcification of the anterior longitudinal
ligaments (arrowheads).
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What is the diagnosis?
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Diagnosis: Apical fibrobullous disease in a patient with ankylosing
spondylitis
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Discussion
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The association of ankylosing spondylitis and pulmonary
disease has been known since the 1940s.1
2
Thoracic
manifestations of ankylosing spondylitis are of two types: upper lobe
fibrobullous disease and chest wall restriction.3
4
The
true incidence of fibrobullous lung disease, the more common
manifestation5
of ankylosing spondylitis, is not known,
but reports range from 1 to 30%.6
7
It is predominantly
seen in adult male subjects with the male-to-female ratio of
50:1.8
The human leukocyte antigen-B27 antigen result is
positive in up to 95% of patients with ankylosing
spondylitis.3
4
6
7
The cause of apical
fibrobullous changes is unknown, but several theories have been
suggested. These include diminished upper lobe ventilation due to chest
wall rigidity,9
altered apical mechanical stress due to
rigid thoracic spine,3
recurrent pulmonary infection due
to impaired cough, and respiratory mechanics as a result of thoracic
rigidity.2
Other theories include prior thoracic
irradiation, repeated aspiration pneumonitis secondary to esophageal
muscle dysfunction,10
and cricoarytenoid joint
disease.11
Unless extensive, apical fibrosis is clinically silent. Symptoms may
include cough, hemoptysis, increased sputum production, and dyspnea.
Patients with fibrocystic disease may develop mycetomas and other
infections, such as atypical mycobacterium. High-resolution
CT12
is frequently helpful to evaluate the lung apices for
the presence of infection and especially mycetoma.
Received for publication February 29, 2000.
Accepted for publication March 28, 2000.
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References
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Dunham, CL, Kautz, FG (1941) Spondylarthritis ankylopoietica: a review and report of twenty cases. Am J Med Sci 201,232-250[CrossRef]
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Hamilton, KA (1949) Pulmonary disease manifestations of ankylosing spondylarthritis. Ann Intern Med 31,216-227[Medline]
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Tanoue, LT (1992) Pulmonary involvement in collagen vascular disease: a review of the pulmonary manifestations of the Marfan syndrome, ankylosing spondylitis, Sjogren’s syndrome and relapsing polychondritis. J Thorac Imaging 7,62-77[CrossRef][ISI][Medline]
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Wiedemann, HP, Matthay, RA (1989) Pulmonary manifestations of the collagen vascular diseases. Clin Chest Med 10,677-722[ISI][Medline]
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Rosenow, E, Strimlan, CV, Muhm, JR, et al (1977) Pleuropulmonary manifestations of ankylosing spondylitis. Mayo Clin Proc 52,641-649[ISI][Medline]
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Hunninghake, GW, Fauci, AS (1979) Pulmonary involvement in the collagen vascular diseases. Am Rev Respir Dis 119,471-503[ISI][Medline]
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Rumancik, WM, Firooznia, H, Davis, MS, et al (1984) Fibrobullous disease of the upper lobes: an extraskeletal manifestation of ankylosing spondylitis. J Comput Tomogr 8,225-229[CrossRef][Medline]
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Boulware, DW, Weissman, DN, Doll, NJ (1985) Pulmonary manifestations of rheumatic diseases. Clin Rev Allergy Immunol 3,249-267
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Stewart, RM, Ridyard, JB, Pearson, JD (1976) Regional lung function in ankylosing spondylitis. Thorax 31,433-437[Abstract]
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Scobie, BA (1970) Disturbed esophageal manometric responses in patients with ankylosing spondylitis and pulmonary aspergillomas. Aust Ann Med 19,131-134
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Libby, DM, Schley, WS, Smith, JP (1981) Cricoarytenoid arthritis in ankylosing spondylitis: a cause of acute respiratory failure and cor pulmonale. Chest 80,641-643[Abstract/Free Full Text]
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Fenlon, HM, Casserley, I, Sant, SM, et al (1997) Plain radiographs and thoracic high-resolution CT in patients with ankylosing spondylitis. AJR Am J Roentgenol 168,1067-1072[Abstract/Free Full Text]
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Introduction
What is the diagnosis?
