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Published on: June 16, 2020
Diffuse alveolar damage: uncommon manifestation of pulmonary involvement in patients with connective tissue diseases
Joseph G Parambil1, Jeffrey L Myers, Jay H Ryu
1Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN 55905, USA.
Insights
Diffuse alveolar damage (DAD) in connective tissue disease (CTD) patients can complicate existing interstitial lung disease (ILD) or present as a new CTD diagnosis. Outcomes are worse for those with prior ILD.
Area of Science:
- Pulmonary Medicine
- Rheumatology
- Pathology
Background:
- Diffuse alveolar damage (DAD) is a frequent finding in surgical lung biopsies, with diverse potential etiologies.
- Connective tissue diseases (CTDs) are known to be associated with various pulmonary complications, including interstitial lung disease (ILD).
Purpose of the Study:
- To investigate the association between connective tissue disease (CTD) and diffuse alveolar damage (DAD) diagnosed via surgical lung biopsy.
- To explore the clinical implications and outcomes of DAD in patients with CTDs.
Main Methods:
- Retrospective analysis of nine consecutive patients with CTD and DAD diagnosed on surgical lung biopsy.
- Review of patient demographics, underlying CTDs, clinical presentation, imaging findings (CT chest), and treatment outcomes.
Main Results:
- The study cohort included patients with rheumatoid arthritis, polymyositis, systemic sclerosis, and mixed CTD.
- DAD occurred either as a complication of pre-existing CTD-related ILD (78% of cases) or as an initial manifestation leading to CTD diagnosis (22% of cases).
- Hospital survival was 44%, with a poorer prognosis observed in patients with pre-existing chronic ILD compared to those without.
Conclusions:
- Diffuse alveolar damage (DAD) is a significant complication in patients with connective tissue disease (CTD)-related interstitial lung disease (ILD).
- DAD can also serve as an early presenting sign of an undiagnosed CTD.
- The presence of pre-existing chronic ILD in CTD patients with DAD is associated with a worse clinical outcome.
Background:
Diffuse alveolar damage (DAD) is a relatively common finding on surgical lung biopsy and can result from a variety of causes.
Methods:
We studied nine consecutive patients with connective tissue disease (CTD) and DAD diagnosed on surgical lung biopsy to examine this association and clinical implications.
Results:
The median age was 63 years (range, 35 to 76 years), and seven of the patients were women (78%). Underlying CTDs included rheumatoid arthritis in five patients, polymyositis in two patients, and one patient each with systemic sclerosis and mixed CTD. In seven patients (78%), CTD had been diagnosed before the onset of DAD; six of these patients had a preexisting interstitial lung disease (ILD) related to their CTD. DAD was the presenting manifestation leading to a new CTD diagnosis in two patients (22%). CT of the chest revealed ground-glass opacities and/or consolidation bilaterally with or without honeycombing. In all patients, surgical lung biopsy revealed DAD for which no cause could be identified other than the underlying CTD. Seven patients (78%) were receiving mechanical ventilatory support at the time of the surgical lung biopsy. Four patients (44%) survived to hospital discharge and included one patient with preexisting ILD and all three patients without chronic ILD.
Conclusion:
We conclude that DAD can complicate the clinical course of patients with CTD-related chronic ILD, or can occasionally occur as a presenting manifestation of CTDs. When DAD occurs in patients with CTDs, the outcome appears to be worse for those with preexisting chronic ILD compared to those without ILD.
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