In this case-control study, HTLV-1 infection increased risk of bronchiectasis 1.84 times. HTLV-1 proviral loads for bronchiectasis patients were significantly higher than those of controls. HTLV-1 proviral loads correlated with the extent of radiologically determined pulmonary injury.
The human T-cell leukaemia virus type 1 (HTLV-1) is associated with pulmonary inflammation. Indigenous Australians in central Australia have a very high prevalence of HTLV-1 infection and we hypothesised that this might contribute to high rates of bronchiectasis in this population.80 Indigenous adults with confirmed bronchiectasis, each matched by age, sex and language to two controls without bronchiectasis, were recruited. Case notes and chest imaging were reviewed, HTLV-1 serology and the number of peripheral blood leukocytes (PBLs) infected with HTLV-1 ( pro-viral load (PVL)) were determined, and radiological abnormality scores were calculated. Participants were followed for a mean±SD of 1.14±0.86 years and causes of death were determined.Median (interquartile range) HTLV-1 PVL for cases was 8-fold higher than controls (cases 213.8 (19.7-3776.3) copies per 10 5 PBLs versus controls 26.6 (0.9-361) copies per 10 5 PBLs; p=0.002). Radiological abnormality scores were higher for cases with HTLV-1 PVL ⩾1000 copies per 10 5 PBLs and no cause of bronchiectasis other than HTLV-1 infection. Major predictors of bronchiectasis were prior severe lower respiratory tract infection (adjusted OR (aOR) 17.83, 95% CI 4.51-70.49; p<0.001) and an HTLV-1 PVL ⩾1000 copies per 10 5 PBLs (aOR 12.41, 95% CI 3.84-40.15; p<0.001). Bronchiectasis (aOR 4.27, 95% CI 2.04-8.94; p<0.001) and HTLV-1 PVL ⩾1000 copies per 10 5 PBLs (aOR 3.69, 95% CI 1.11-12.27; p=0.033) predicted death.High HTLV-1 PVLs are associated with bronchiectasis and with more extensive radiological abnormalities, which may result from HTLV-1-mediated airway inflammation.@ERSpublications Higher numbers of HTLV-1-infected cells in peripheral blood are associated with bronchiectasis and more extensive radiological abnormalities among those with no cause for bronchiectasis other than HTLV-1 infection
BACKGROUND
Chiari Type I malformation (CM1) is a disorder recognized by caudal displacement of the cerebellar tonsils through the foramen magnum and into the cervical canal. Syringomyelia is frequently found in patients with CM1, but the pathophysiology of syringomyelia remains an enigma. As a general consensus, symptomatic patients should be treated and asymptomatic patients without a syrinx should not be treated. Mildly symptomatic patients or asymptomatic patients with a syrinx, on the other hand, pose a more challenging dilemma, as the natural evolution is uncertain. For many surgeons, the presence of a syrinx is an indication to offer surgery even if the patient is asymptomatic or mildly symptomatic.
OBSERVATIONS
The authors describe an illustrative case of a 31-year-old female with an incidental finding of a CM1 malformation and cervical syrinx in 2013. Conservative management was advocated as the patient was asymptomatic. Monitoring of the syrinx over a course of 8 years showed resolution, followed by reappearance and finally a complete resolution in 2021. A review of the literature and the possible pathophysiology is discussed.
LESSONS
The unusual course of this patient highlights the importance of guiding treatment by clinical symptoms, not radiological findings. Furthermore it reflects the complexity of the pathophysiology and the uncertain natural history of syringomyelia.
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