Pemphigus vulgaris-associated interstitial lung disease

Abstract

Autoimmune bullous diseases (AIBDs)-associated interstitial lung disease (ILD) is extremely rare. Pemphigus vulgaris (PV) is an intraepidermal autoimmune blistering disease caused by circulating autoantibodies against desmoglein. To date, PV-associated ILD has rarely been reported in English literature. We report a rare association of PV and ILD. A 53-year-old Chinese female with PV for 8 months developed ILD after a relapse of PV for 2 months due to discontinuation of oral prednisone by herself. She was successfully treated by systemic methylprednisolone. Taken previously reported bullous pemphigoid-associated ILD and linear IgA/IgG bullous dermatosis-associated ILD together, in general, AIBDs-associated ILD occurs when AIBDs relapse or are not controlled, responds well to systemic corticosteroids, and has a relatively better prognosis when compared with rheumatoid arthritis- or dermatomyositis-associated ILD.

A case of bullous pemphigoid associated with interstitial pneumonia

“…The reason for obscure direct immunofluorescence testing results in our case may be that few stem cells expressed BP180. Previous reports of autoimmune bullous diseases complicated by interstitial pneumonia supports our speculation.…”

Dermatomyositis in patients with autoimmune blistering diseases

“…AIBD-associated interstitial lung disease (ILD) is rare, and linked with relapse and treatment-resistant AIBD (Usuba et al., 1989). In these cases, ILD has an improved response to systemic corticosteroids and a relatively better prognosis when compared with RA- or DM-associated ILD (Bai et al., 2016, Yoshioka et al., 2012).…”

Pulmonary interstitial lesions in pemphigus mouse model: Verifying pemphigus may not be only limited to skin and mucosa

“…3 However, in recent years, larger cross-sectional studies of pemphigus had found that thyroid diseases, rheumatoid arthritis, alopecia areata and other diseases were also associated with pemphigus, among which interstitial lung disease (ILD) was particularly noteworthy for its high mortality. [4][5][6][7][8] Through the statistical analysis of 283 patients with pemphigus, our group found that ILD was the most common complication in patients with pemphigus, except for steroid-related complications,…”