Xerotrachea and Interstitial Lung Disease in Primary Sjogren’s Syndrome

Abstract

22 patients with primary Sjogren’s syndrome were prospectively studied for respiratory system involvement with clinical, roentgenological and functional parameters. 12 patients (55%) had respiratory manifestations. In 10/12 (83%), respiratory symptoms occurred before or concomitantly with the classical symptoms of Sjogren’s syndrome. There were two distinct forms of respiratory involvement: xerotrachea, manifested by dry cough without other symptoms and negative roentgenological and functional evaluation and diffuse interstitial lung disease manifested by dyspnea with or without dry cough with bibasilar rales, compatible chest roentgenogram, restrictive pattern in spirometry and/or hypoxemia. Xerotrachea was more common in patients with glandular form of Sjogren’s syndrome (3/10) and diffuse interstitial lung disease in patients with extraglandular form (6/12). None of the 22 patients had pleurisy or other forms of respiratory involvement. Antibodies to Ro (SSA) and La (SSB) antigens were more common in patients with the extraglandular form of the syndrome but did not correlate with diffuse interstitial lung disease

Lung involvement in primary Sjogren's syndrome is mainly related to the small airway disease

“…Most patients suffer only from dry cough without specific clinical findings. Dry cough has been described by Constantopoulos et al 3 as being the main respiratory symptom of primary Sjögren’s syndrome patients and has been named “xerotrachea” following the equivalence of xerostomia and xerophthalmia. Dry cough has been described also by Sjögren himself 65 years ago, later named “bronchitis sicca” by Alarcon-Segovia 12.…”

Thin-section chest CT findings of primary Sjögren's syndrome: correlation with pulmonary function

“…6a, b Transverse thin-section CT scans of the lungs from a 76-year-old woman with PSS and ILF shows diffuse ground-glass attenuation with multiple randomly distributed thin-walled lung cysts. Mild septal and intralobular thickening is also present The results are expressed as percentages of the normal predicted values (mean±SD) The 2 patients with normal scans and the 2 patients with findings or images suggestive of pulmonary hypertension were excluded PSS patients [5,10,34], leading to an increased risk of recurrent respiratory tract infections in association with tracheal and bronchial dryness [4,35], Airway disease is defined as bronchial wall thickening and/or bronchiectasis when the large airways are involved and findings of cellular and constrictive bronchiolitis when the small airways are affected [36]. Franquet et al reported CT abnormalities of the small airways in 11 of 34 (32%) of patients [34].…”

Lung function in primary Sjogren's syndrome: a cross sectional and longitudinal study.

“…REVIEW In 30 of the earliest patients to be fully documented in the clinic the assessment of symptoms and signs of possible chest disease was repeated after a mean interval of four years. Any change in smoking habit was Lung function in primary Sjdgren's syndrome: a cross sectional and longitudinal study Table I Diagnostic criteria usedfor primary Sjogren's syndrome (at least three necessary for diagnosis) * 1 Symptomatic xerostomia 2 Abnormal response to Schirmer's test (< 10 mm wetting in 5 min bilaterally) 3 Positive result in serological tests (antinuclear factor or rheumatoid factor > 1/40) or " + " anti-Ro antibody 4 Positive lip biopsy specimen (grades II, III, or IV) *Exclusions include any patients with pre-existent rheumatoid arthritis, systemic lupus erythematosus, sarcoidosis, or lymphoma. recorded.…”