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connectivitis-associated lung diseases

Sunday 29 March 2020

connective tissue disease-associated lung diseases.

Connective tissue diseases (CTDs) can affect all compartments of the lungs, including airways, alveoli, interstitium, vessels, and pleura.

CTD-associated lung diseases (CTD-LDs) may present as diffuse lung disease or as focal lesions, and there is significant heterogeneity between the individual CTDs in their clinical and pathological manifestations.

CTD-LDs may presage the clinical diagnosis a primary CTD, or it may develop in the context of an established CTD diagnosis.

CTD-LDs reveal acute, chronic or mixed pattern of lung and pleural manifestations.

Histopathological findings of diverse morphological changes can be present in CTD-LDs :
- airway lesions (chronic bronchitis/bronchiolitis, follicular bronchiolitis, etc.),
- interstitial lung diseases (nonspecific interstitial pneumonia/fibrosis, usual interstitial pneumonia, lymphocytic interstitial pneumonia, diffuse alveolar damage, and organizing pneumonia),
- pleural changes (acute fibrinous or chronic fibrous pleuritis),
- vascular changes (vasculitis, capillaritis, pulmonary hemorrhage, etc.).

CTD patients can be exposed to various infectious diseases when taking immunosuppressive drugs.

Histopathological patterns of CTD-LDs are generally nonspecific, and other diseases that can cause similar lesions in the lungs must be considered before the diagnosis of CTD-LDs.

A multidisciplinary team involving pathologists, clinicians, and radiologists can adequately make a proper diagnosis of CTD-LDs.

Open references

- Pathological interpretation of connective tissue disease-associated lung diseases. Kwon KY. Yeungnam Univ J Med. 2019 Jan;36(1):8-15. doi : 10.12701/yujm.2019.00101 PMID: 31620606 Free