Interstitial Pneumonia with Autoimmune Features: Why Rheumatologist-Pulmonologist Collaboration Is Essential

Sebastiani, Marco; Faverio, Paola; Manfredi, Andreina; Cassone, Giulia; Vacchi, Caterina; Stainer, Anna; Pozzi, Maria Rosa; Salvarani, Carlo; Pesci, Alberto; Luppi, Fabrizio

doi:10.3390/biomedicines9010017

Cited by 16 publications

(15 citation statements)

References 73 publications

(43 reference statements)

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“…The decision on how far to search for evidence of an underlying CTD often impacts the eventual diagnosis [32]. The current IPAF criteria do not specify a need for specialist rheumatologist evaluation, but the data summarised above suggest that rheumatological input might help to improve the sensitivity and specificity of the IPAF criteria [33].…”

Section: Inclusion Of Rheumatological Reviewmentioning

confidence: 99%

Interstitial pneumonia with autoimmune features: challenges and controversies

et al. 2021

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The presence of clinical, serological and/or radiological features suggestive, but not confirmatory, of a defined connective tissue disease in patients with interstitial lung disease is a relatively frequent occurrence. In 2015, the European Respiratory Society and the American Thoracic Society proposed classification criteria for the interstitial pneumonia with autoimmune features (IPAF) research entity to capture such patients in a standardised manner, with the intention of nurturing clinical research. This initiative resulted in the publication of several series of IPAF patients, with significant variation between cohorts in clinical characteristics, outcome and the application of IPAF criteria in patient selection. From this increasing body of published work, it has become apparent that revision of IPAF criteria is now required in order to justify the eventual designation of IPAF as a standalone diagnostic term, as opposed to a provisional entity put forward as a basis for clinical research. This review covers the current state of IPAF, conclusions that can and cannot be drawn from the IPAF evidence base, and ongoing uncertainties that require further expert group consideration.

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Section: Inclusion Of Rheumatological Reviewmentioning

confidence: 99%

Interstitial pneumonia with autoimmune features: challenges and controversies

et al. 2021

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“…Further, once the patient progresses to pulmonary fibrosis, the prognosis becomes less optimistic. Owing to the complexity of treatment, tailoring treatment protocols for CTD-ILD requires vigorous effort and a multidisciplinary team approach often including close collaboration with the patient’s pulmonologist ( 4 , 5 ).…”

Section: Introductionmentioning

confidence: 99%

Interstitial Lung Disease in Connective Tissue Disease: A Common Lesion With Heterogeneous Mechanisms and Treatment Considerations

et al. 2021

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Connective tissue disease (CTD) related interstitial lung disease (CTD-ILD) is one of the leading causes of morbidity and mortality of CTD. Clinically, CTD-ILD is highly heterogenous and involves rheumatic immunity and multiple manifestations of respiratory complications affecting the airways, vessels, lung parenchyma, pleura, and respiratory muscles. The major pathological features of CTD are chronic inflammation of blood vessels and connective tissues, which can affect any organ leading to multi-system damage. The human lung is particularly vulnerable to such damage because anatomically it is abundant with collagen and blood vessels. The complex etiology of CTD-ILD includes genetic risks, epigenetic changes, and dysregulated immunity, which interact leading to disease under various ill-defined environmental triggers. CTD-ILD exhibits a broad spectra of clinical manifestations: from asymptomatic to severe dyspnea; from single-organ respiratory system involvement to multi-organ involvement. The disease course is also featured by remissions and relapses. It can range from stability or slow progression over several years to rapid deterioration. It can also present clinically as highly progressive from the initial onset of disease. Currently, the diagnosis of CTD-ILD is primarily based on distinct pathology subtype(s), imaging, as well as related CTD and autoantibodies profiles. Meticulous comprehensive clinical and laboratory assessment to improve the diagnostic process and management strategies are much needed. In this review, we focus on examining the pathogenesis of CTD-ILD with respect to genetics, environmental factors, and immunological factors. We also discuss the current state of knowledge and elaborate on the clinical characteristics of CTD-ILD, distinct pathohistological subtypes, imaging features, and related autoantibodies. Furthermore, we comment on the identification of high-risk patients and address how to stratify patients for precision medicine management approaches.

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“…ILD occurs in most patients with AAV at the same time (36 -67%) as or before the onset of vasculitis (14 -85%) 19 . It is not common for the onset of AAV to precede the diagnosis of ILD (8 -21%).…”

Section: Anca-positive Idiopathic Interstitial Pneumoniamentioning

confidence: 99%

“…In particular, ILD can initially manifest as ANCA-positive idiopathic interstitial pneumonia (IIP) and overt vasculitis can develop months to 12 years later 11 . The prevalence of MPO-ANCA in patients with IIP ranges from 4 to 35%, whereas PR3-ANCA is rare (2 -4%) 19 . Patients with ANCA-positive IIP usually present with symptoms of shortness of breath or cough, whereas hemoptysis and constitutional symptoms are less frequent.…”

Section: Anca-positive Idiopathic Interstitial Pneumoniamentioning

confidence: 99%

Interstitial Lung Disease and Diffuse Alveolar Hemorrhage, the Two Key Pulmonary Manifestations in Microscopic Polyangiitis

Kim¹,

Lee²

2021

Tuberc Respir Dis

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Microscopic polyangiitis (MPA) is an antineutrophil cytoplasmic antibody (ANCA)‒associated necrotizing vasculitis, which mainly affects small vessels in various organs, especially the lungs. The two key pulmonary manifestations, interstitial lung disease (ILD) and diffuse alveolar hemorrhage (DAH), increase the morbidity and death rate of patients with MPA. ILD is more common in MPA than in other ANCA-associated vasculitis subsets and is primarily associated with myeloperoxidase-ANCA. Unlike alveolar hemorrhage due to pulmonary capillaritis, ILD can initially manifest as isolated pulmonary fibrosis. Of note, its most frequent radiographic pattern is the usual interstitial pneumonia pattern, similar to the characteristic pattern seen in idiopathic pulmonary fibrosis. In this review we present the pathogenesis, clinical manifestations, and radiographic and histopathologic features of ILD and DAH in MPA. We also briefly summarize the outcome and therapeutic options for the two conditions.

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Interstitial Pneumonia with Autoimmune Features: Why Rheumatologist-Pulmonologist Collaboration Is Essential

Cited by 16 publications

References 73 publications

Interstitial pneumonia with autoimmune features: challenges and controversies

Interstitial pneumonia with autoimmune features: challenges and controversies

Interstitial Lung Disease in Connective Tissue Disease: A Common Lesion With Heterogeneous Mechanisms and Treatment Considerations

Interstitial Lung Disease and Diffuse Alveolar Hemorrhage, the Two Key Pulmonary Manifestations in Microscopic Polyangiitis

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