"Usual" interstitial pneumonia with autoimmune features: a prospective study on a cohort of idiopathic pulmonary fibrosis patients

Sambataro, Gianluca; Ferrara, C.; Torrisi, Sebastiano Emanuele; Spadaro, Carla; Vignigni, Giovanna; Vancheri, Ada; Papa, Nicoletta Del; Orlandi, Martina; Colaci, Michele; Malatino, Lorenzo; Palmucci, Stefano; Cavagna, Lorenzo; Sambataro, Domenico; Vancheri, Carlo

doi:10.55563/clinexprheumatol/lqi6z7

Cited by 8 publications

(10 citation statements)

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“…Therefore, ILD patients with a UIP pattern associated with only a clinical or a serological domain were considered IPF; however, in our internal classification, they are considered “UIPAF” and are followed for possible progression. We have already demonstrated that “UIPAF” patients progress towards a specific CTD in a greater proportion than IPF patients, similarly to classic IPAF [25, 26]. In this study, IPAF and “UIPAF” are considered together, which could explain why the proportion of ILD-PU patients that progressed towards a specific CTD is higher than that reported in the literature.…”

Section: Discussionmentioning

confidence: 67%

Clinical Presentation of Connective Tissue Disease Patients with and without Interstitial Lung Disease: A Retrospective Study

Sambataro¹,

Libra²,

Spicuzza³

et al. 2023

Respiration

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Background: Connective tissue diseases (CTDs) are responsible for about 20% of interstitial lung disease (ILD) cases, but their diagnosis in a pulmonary unit (PU) is not always straightforward due to a heterogeneous clinical picture. Objectives: The aim of this study was to evaluate the clinical presentation of rheumatoid arthritis (RA) and CTD-ILD cases diagnosed in PU, compared to RA and CTD patients diagnosed in a rheumatologic unit (RU). Methods: Patients with RA, systemic sclerosis (SSc), primary Sjӧgren’s syndrome (pSS), and idiopathic inflammatory myopathy were retrospectively enrolled from an RU and a PU designated to manage ILD during a period from January 2017 to October 2022. The classification of CTD-PU was carried out in a multidisciplinary setting, including the same rheumatologists that diagnosed CTD in the RU. Results: ILD-CTD-PU patients were prevalently male and older. Progression from undifferentiated CTD to a specific condition was more common in ILD-CTD-PU, and those patients generally obtained a lower score on specific classification criteria. RA-PU patients resembled polymyalgia rheumatica in 47.6% of cases, also showing a greater proportion of typical joint deformities (p = 0.02). SSc-PU patients showed a usual interstitial pneumonia pattern in 76% of cases and, compared with SSc-RU, were more commonly seronegative (p = 0.03) and generally lacked fingertip lesions (p = 0.02). The majority of the diagnoses of pSS-PU were in patients with previously diagnosed ILD, in which seropositivity and sicca syndrome developed during follow-up. Conclusions: CTD-ILD patients diagnosed in the PU show severe lung involvement and a nuanced autoimmune clinical picture.

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Section: Discussionmentioning

confidence: 67%

Clinical Presentation of Connective Tissue Disease Patients with and without Interstitial Lung Disease: A Retrospective Study

Sambataro¹,

Libra²,

Spicuzza³

et al. 2023

Respiration

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“…In summary, we provide a thorough description of the characteristics of patients with autoimmune IP from a real-world cohort, illustrating that the assessment of the individual IPAF defining items can help identify homogeneous subgroups of the disease beyond the diagnostic classification. This segregation is necessary in order to advance to a precision-based medicine in this complex field ( 35 ). In addition, while some clinical features appear to be under-represented in patients without an overt underlying CTD, performance of NVC should be encouraged since it might help improve current IPAF definition and further distinguish 2 types of disease according to the presence or absence of autoimmune vasculopathy.…”

Section: Discussionmentioning

confidence: 99%

Fine-tuning characterization of patients with interstitial pneumonia and an underlying autoimmune disease in real-world practice: We get closer with Nailfold videocapillaroscopy

Romero-Bueno

Rodríguez-Nieto²,

Miras³

et al. 2023

Front. Med.

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ObjectivesTo assess performance of interstitial pneumonia (IP) with autoimmune features (IPAF) criteria in clinical practice and describe the utility of additional workup in identifying patients with underlying connective tissue diseases (CTD).MethodsWe set a retrospective study of our patients with autoimmune IP, who were allocated to CTD-IP, IPAF or undifferentiated autoimmune IP (uAIP) subgroups according to the updated classification criteria. Presence of the process-related variables comprising IPAF defining domains was scrutinized in all patients, and, when available, the results of nailfold videocapillaroscopy (NVC) were recorded.ResultsThirty nine out of 118 patients, accounting for 71% of former undifferentiated cases, fulfilled IPAF criteria. Arthritis and Raynaud’s phenomenon were prevalent in this subgroup. While systemic sclerosis-specific autoantibodies were restricted to CTD-IP patients, anti-tRNA synthetase antibodies were also present in IPAF. In contrast, rheumatoid factor, anti-Ro antibodies and ANA nucleolar patterns could be found in all subgroups. Usual interstitial pneumonia (UIP) / possible UIP were the most frequently observed radiographic patterns Therefore, the presence of thoracic multicompartimental findings as also performance of open lung biopsies were useful in characterizing as IPAF those UIP cases lacking a clinical domain. Interestingly, we could observe NVC abnormalities in 54% of IPAF and 36% of uAIP tested patients, even though many of them did not report Raynaud’s phenomenon.ConclusionBesides application of IPAF criteria, distribution of IPAF defining variables along with NVC exams help identify more homogeneous phenotypic subgroups of autoimmune IP of potential relevance beyond clinical diagnosis.

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“…32 Studies on ILD associated with undifferentiated CTD have not proved an evolution towards MCTD, thus further supporting the idea that ILD is present in longstanding disease, or at least when the clinical picture is sufficient to reach a specific classification as MCTD. [54][55][56] HRCT can be also useful in evaluating possible pulmonary artery enlargement, as a clue to a possible, underlying PAH, which is relatively common in ILD-MCTD patients. 57 Appropriate screening for PAH can be carried out with a transthoracic echocardiogram, which should be considered a first-line exam in the assessment of MCTD patients.…”

Section: Instrumental Evaluationmentioning

confidence: 99%

Towards Early Diagnosis of Mixed Connective Tissue Disease: Updated Perspectives

Ferrara¹,

Rocca²,

Ielo³

et al. 2023

ITT

Self Cite

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Mixed Connective Tissue Disease (MCTD) is an autoimmune disease first described by Sharp et al in 1972, characterized by the presence of anti-Ribonucleoprotein antibodies directed against the U1 complex (anti-U1RNP). The condition shares clinical characteristics with Systemic Lupus Erythematosus, Rheumatoid Arthritis, and Systemic Sclerosis. Diagnosis is quite difficult due to its rarity, the lack of validated classification criteria, and its heterogeneous clinical presentation. While in the early stages its nuanced clinical features might lead to it being incorrectly classified as other Connective Tissue Diseases (CTDs) or even not recognized, in cases of longstanding disease its classification as a CTD is clear but challenging to discriminate from overlap syndromes. MCTD should be considered a distinct entity due to the presence of a specific genetic substrate and the presence of the high titer of a specific autoantibody, anti-U1RNP, present in all the commercial kits for Extractable Nuclear Antigens, and almost always associated with Antinuclear Antibody positivity with a coarse speckled pattern. Except for anti-U1RNP, no specific biomarkers are available to guide clinicians to a correct classification of MCTD, which is arrived at by the association of clinical, serological and instrumental evaluation. In the first stages, the disease is mainly characterized by Raynaud’s phenomenon, inflammatory arthritis, puffy fingers, myalgia and/or myositis, and rarely, trigeminal neuropathy. Longstanding disease is generally associated with the development of Pulmonary Hypertension and Interstitial Lung Disease, which are the two main causes of mortality in MCTD. The aim of this review is to summarize current knowledge on the early recognition of MCTD.

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"Usual" interstitial pneumonia with autoimmune features: a prospective study on a cohort of idiopathic pulmonary fibrosis patients

Cited by 8 publications

References 0 publications

Clinical Presentation of Connective Tissue Disease Patients with and without Interstitial Lung Disease: A Retrospective Study

Clinical Presentation of Connective Tissue Disease Patients with and without Interstitial Lung Disease: A Retrospective Study

Fine-tuning characterization of patients with interstitial pneumonia and an underlying autoimmune disease in real-world practice: We get closer with Nailfold videocapillaroscopy

Towards Early Diagnosis of Mixed Connective Tissue Disease: Updated Perspectives

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