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Alessia Alunno [1] and Roberto Gerli [1] and Roberto Giacomelli [2] and Francesco Carubbi [2; 3]
Academic Editor: Dimitrios P. Bogdanos
1, Rheumatology Unit, Department of Medicine, University of Perugia, Perugia, Italy, unipg.it
2, Rheumatology Unit, Department of Biotechnological and Applied Clinical Science, School of Medicine, University of L’Aquila, L’Aquila, Italy, univaq.it
3, Department of Medicine, ASL 1 Avezzano Sulmona L’Aquila, L’Aquila, Italy, asl1abruzzo.it
Received Jun 29, 2017; Revised Aug 22, 2017; Accepted Oct 10, 2017
This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
1. Introduction
Rheumatoid arthritis (RA) is a chronic inflammatory disease of the musculoskeletal system with a clinical picture dominated by signs and symptoms of joint involvement [1]. The hallmark of the disease is symmetric chronic synovitis mainly affecting small peripheral joints, but virtually all joints equipped with a synovial membrane can be involved. Although this inflammatory process is reversible, it may evolve into irreversible damage of articular structures if left untreated and may lead to loss of function [2]. Although RA is by definition an articular disorder, it is a systemic disease. In fact, in a consistent subgroup of patients, extra-articular manifestations (EAMs) may occur, affecting other organs and tissues and thereby worsening disease prognosis. The incidence of EAMs is widely variable across different studies. Overall, EAMs occur in 18–40% of RA patients, while the incidence of severe EAMs ranges from 1 to 20%. Interestingly, EAMs may often be the first presentation of the disease before any articular involvement. Furthermore, extra-articular sites can also be the target of comorbidities (e.g., cardiovascular (CV) manifestations) or of the damage induced by RA-related drugs (e.g., corticosteroid-induced osteoporosis), requiring therefore an accurate differential diagnosis [3]. The involvement of the respiratory system occurs in about 30–40% of RA patients, and it is the first manifestation of the disease in about 10–20% of them (Figure 1). Pulmonary manifestations in RA mainly reflect the involvement of interstitium, airways, and pleurae, while vascular involvement is less frequent and diffuse alveolar hemorrhage (DAH), when observed in these patients, has a different etiology [4]. Overall, pulmonary manifestations account for 10–20% of mortalities in RA [5] and have been...