Yeungnam Univ J Med.  2019 Jan;36(1):8-15. 10.12701/yujm.2019.00101.

Pathological interpretation of connective tissue disease-associated lung diseases

• Kwon KY

• KMID: 2434093
• DOI: http://doi.org/10.12701/yujm.2019.00101

Abstract

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), and 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.

Keyword

Airway diseases; Connective tissue diseases; Histopathology; Interstitial lung diseases; Pleural diseases

MeSH Terms

Bronchiolitis
Communicable Diseases
Connective Tissue Diseases
Connective Tissue*
Diagnosis
Fibrin
Hemorrhage
Humans
Idiopathic Pulmonary Fibrosis
Lung Diseases*
Lung Diseases, Interstitial
Lung*
Pleura
Pleural Diseases
Population Characteristics
Fibrin

Figure

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  Fig. 1. Chronic fibrous pleuritis in a patient with rheumatoid arthritis. Pleural adhesion (black arrows), fibrotic interstitial pneumonitis (asterisks) and focally lymphoid aggregates (white arrow) are present (hematoxylin and eosin stain, ×40).

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  Fig. 2. Usual interstitial pneumonia pattern with lymphoid hyperplasia in a patient with rheumatoid arthritis. Microscopic findings of honeycombing (black arrows) and lymphoid follicle (white arrow) are seen. Fibrous pleural thickening is noted (arrow heads) (hematoxylin and eosin stain, ×40).

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  Fig. 3. Fibrotic NSIP in a patient with systemic sclerosis. Diffuse fibrotic interstitial thickening with NSIP pattern and relatively sparing the subpleural area (A, arrows heads). The thickened interstitium shows paucicellular and collagenous fibrosis (B, arrows) (hematoxylin and eosin stain, ×10 [A] and ×200 [B]). NSIP, nonspecific interstitial pneumonia.

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  Fig. 4. Lung parenchymal changes in a patient with systemic lupus erythematosus. Histological patterns of organizing diffuse alveolar damage (A) and organizing pneumonia (arrows) (B) are present (hematoxylin and eosin stain, ×40 [A] and [B]).

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  Fig. 5. Pulmonary parenchymal hemorrhage in a patient with systemic lupus erythematosus. (A) Diffuse parenchymal hemorrhage associated with lymphoid aggregates (arrow) and vascular congestion in the fibrotic interstitium. (B) Diffuse and fresh alveolar hemorrhage (arrows) with no distinct capillaritis (hematoxylin and eosin stain, ×40 [A] and ×100 [B]).

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  Fig. 6. Fibrotic nonspecific interstitial pneumonia pattern in a patient with polymyositis (A). Usual interstitial pneumonia pattern is present in another lesion of the same patient (B). Diffuse interstitial fibrotic thickening associated with honeycombing (asterisk) and scattered lymphoid aggregates (arrows) are present (hematoxylin and eosin stain, ×10 [A] and ×100 [B]).

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  Fig. 7. Lymphocytic interstitial pneumonia pattern in a patient with Sjögren syndrome. (A, B) Various degree of the interstitial thickening with abundant lymphoplasmacytic cells aggregates (arrows) is present (hematoxylin and eosin stain, ×40 [A] and ×100 [B]). Courtesy of Professor Jin-Haeng Chung, M.D., Seoul National University Bundang Hospital.

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