connectivitis-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