Introduction

The lung is a frequent target of autoimmune-mediated injury in patients with connective tissue diseases (CTDs) [1, 2]. Myositis, Systemic Lupus Erythematosus (SLE), Sjögren’s syndrome (SS), and Mixed Connective Tissue Disease (MCTD) can affect different levels of the respiratory tract, with a wide range of symptoms intensity, from asymptomatic to severe or life-threatening forms. Pleural disease is the most common lung manifestation in SLE and MCTD, while SS primarily affects the airways [3]. Myositis mostly affects the parenchyma, and interstitial lung disease (ILD) is the main lung manifestation of this disease [4]. However, ILD can also present in SLE, SS, and MCTD with different clinical presentations, ranging from minimal signi cant pulmonary restriction to severe progressive pulmonary brosis [5, 6].

Lung involvement in CTDs is associated with a poor prognosis, leading to increased morbidity and mortality, and an altered quality of life. Early recognition and treatment are imperative issues to limit morbidity and mortality. In addition, clinicians must be aware of non-speci c pulmonary complications, such as infection, pulmonary embolism, and left heart failure, which may coexist with speci c lung manifestations and contribute to increased morbidity.

Diagnosis of a pulmonary involvement can be easy when occurring in patients with a previously diagnosed CTD or can be helped by the presence of clinical features suggestive of CTD. The close collaboration of pneumologists and rheumatologists is central to the assessment of any patient with a suspicion of CTD, particularly in patients with ILD [7]. Besides clinical expertise, the use of new diagnostic tools, such as ultrasound imaging, where rheumatologists developed speci c expertise, may be useful to identify and characterize CTD in patients with respiratory disorders [8] or to screen for lung involvement in patients with CTD [9]. However, respiratory symptoms might occur before any other symptom, and features of CTD can manifest later during follow-up. Homma et al. observed that 19% of the patients with ILD developed CTDs over a period of one to 11 years [10]. These manifestations could be a direct consequence of the infammatory disease, such as ILD, or an indirect lung involvement such as infections or drug toxicity.

In this review, we aim to provide a short overview of lung involvement of these four CTDs; SLE, SS, MCTD, and myositis. We focus mostly on describing the pulmonary manifestations, the pathophysiology, and the treatment strategies based on current literature.

Mada Ghanem and Eirini Vasarmidi contributed equally with all other contributors.

M. Ghanem · E. Vasarmidi · B. Crestani (*)

APHP, Service de Pneumologie A, Centre de référence des Maladies Pulmonaires Rares, FHU APOLLO, Hôpital Bichat, Paris, France

Université Paris CITÉ, Inserm, U1152, laboratoire d’excellence INFLAMEX, Paris, France

e-mail: bruno.crestani@bch.ap-hop-paris.fr

L. Morer · P. Le Guen

APHP, Service de Pneumologie A, Centre de référence des Maladies Pulmonaires Rares, FHU APOLLO, Hôpital Bichat, Paris, France

Clinical Vignette

A 56-year-old female with no medical history was evaluated for increasing dyspnea over 3 weeks with hands and ankles infammatory pain. Physical examination revealed heliotrope facial edema (Fig. 14.1a) with hands and n- gers swelling (Fig. 14.1b). Bibasal crackles were heard. Blood tests revealed elevated creatine kinase. A chest computed tomography showed bilateral consolidation with ground glass opacities and a localized pneumothorax (Fig. 14.1c, d). Serology detected anti-nuclear antibodies 1/600, with anti-­MDA5 positivity. A diagnosis of dermatomyositis was given. The patient was treated with corticosteroids and intravenous cyclophosphamide.