Titlebook: Orphan Lung Diseases; A Clinical Guide to Vincent Cottin,Luca Richeldi,Francis X. McCormack Textbook 2023Latest edition The Editor(s) (if

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Lung Disease in Systemic Lupus Erythematosus, Myositis, Sjögren’s Disease, and Mixed Connective Tisstion of this disease [4]. However, ILD can also present in SLE, SS, and MCTD with different clinical presentations, ranging from minimal significant pulmonary restriction to severe progressive pulmonary fibrosis [5, 6].

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Interstitial Pneumonia with Autoimmune Features. Her high-resolution computed tomography images reveal evidence of diffuse lung disease suggestive of nonspecific interstitial pneumonia (NSIP) pattern (Fig. 15.1). Laboratory testing is notable only for a positive anti-nuclear antibody at high titer (1:1280). All other serologies and lab tests are normal.

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Orphan Lung Diseases: From Definition to Organization of Caremanagement of their disease, and, as such, hope for the future is dim because so little research has been devoted to understanding such rare disorders in patients and improving their quality of life [1]. Their feeling of abandonment is intensified by comparisons with patients who suffer from common

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Challenges of Clinical Research in Orphan Diseasesly, these conditions are generally poorly studied, frequently not well-understood, and often lack effective therapies. In recent years, the appreciation of the importance of research into rare diseases has grown considerably. In fact, rare disease research may not only benefit these patients directl

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Orphan Tracheopathieslooked in patients presenting with nonspecific respiratory complaints. These tracheopathies include idiopathic subglottic stenosis (ISS), tracheobronchopathia osteochondroplastica (TPO), tracheomalacia (Mounier-Kuhn syndrome), tracheobronchomegaly, and tracheopathies associated with systemic disease

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