PMID- 37654644
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20230902
IS  - 2529-198X (Electronic)
IS  - 2529-198X (Linking)
VI  - 34
IP  - 2
DP  - 2023 Jun
TI  - Clinical and Serological Characteristics of a Monocentric Cohort of Patients 
      Affected by Interstitial Pneumonia with Autoimmune Features (IPAF).
PG  - 180-187
LID - 10.31138/mjr.34.2.180 [doi]
AB  - INTRODUCTION: Interstitial lung diseases (ILDs) are diseases characterised by 
      excessive deposition of collagen matrices in the pulmonary interstitium. Some of 
      them are considered idiopathic (idiopathic pulmonary fibrosis - IPF), others are 
      related to known pathologies such as connective tissue diseases (CTDs-ILD). 
      Patient affected by ILD and features referable to CTD, not satisfying CTD 
      criteria, are called Interstitial pneumonia with autoimmune features (IPAF) 
      patients. OBJECTIVE: The aim of this report was to investigate clinical and 
      serologic features of a monocentric cohort of patients with IPAF. Another 
      objective was to describe the autoantibody profile, clinical features, High 
      Resolution Computerised Tomography (HRCT) and Nailfold Video Capillaroscopy (NVC) 
      patterns. METHODS: 36 IPAF patients were consecutively enrolled. Clinical, 
      serological, and morphological features were collected. RESULTS: 36 consecutive 
      IPAF patients were enrolled from January 2021 to January 2022. Raynaud's 
      phenomenon was the most frequent symptom identified. We also described other 
      signs and symptoms not included in IPAF criteria. 36,1% of patients demonstrated 
      a Usual Interstitial Pneumonia (UIP) pattern by HRCT. Pulmonary arterial pressure 
      estimation (PAPs) resulted elevated (>/= 25 mmHg) in 6 patients. Antinuclear 
      antibodies (ANA) >/= 1/80 was the most frequent autoantibody, followed by anti-Ro, 
      in patients with UIP pattern and Non-Specific Interstitial Pneumonia (NSIP) 
      pattern at HRCT. NVC highlighted non-specific microangiopathy as the most common 
      pattern especially in UIP patients. CONCLUSIONS: This paper may contribute to 
      stimulate the interest in better characterisation of clinical, serologic, and 
      instrumental features for IPAF patients by redefining IPAF classification 
      criteria in order to treat them as best as possible.
CI  - (c) 2023 The Mediterranean Journal of Rheumatology (MJR).
FAU - Canofari, Claudia
AU  - Canofari C
AD  - Rheumatology Unit, Az. Osp. San Camillo-Forlanini Hospital Rome, Italy.
FAU - Vendola, Andrea
AU  - Vendola A
AD  - Rheumatology, Allergology and Clinical Immunology, Department of Systems 
      Medicine, University of Rome Tor Vergata, Rome, Italy.
FAU - Iuliano, Annamaria
AU  - Iuliano A
AD  - Rheumatology Unit, Az. Osp. San Camillo-Forlanini Hospital Rome, Italy.
FAU - Di Michele, Loreta
AU  - Di Michele L
AD  - Pulmonary Interstitial Diseases Unit, UOSD interstiziopatie polmonari, Az Osp. 
      San Camillo-Forlanini Hospital, Rome, Italy.
FAU - Sebastiani, Alfredo
AU  - Sebastiani A
AD  - Pulmonary Interstitial Diseases Unit, UOSD interstiziopatie polmonari, Az Osp. 
      San Camillo-Forlanini Hospital, Rome, Italy.
FAU - Gubbiotti, Andreina
AU  - Gubbiotti A
AD  - Clinical Pathology Unit, Az. Osp. San Camillo-Forlanini Hospital, Rome, Italy.
FAU - Sebastiani, Gian Domenico
AU  - Sebastiani GD
AD  - Rheumatology Unit, Az. Osp. San Camillo-Forlanini Hospital Rome, Italy.
LA  - eng
PT  - Journal Article
DEP - 20230630
PL  - Greece
TA  - Mediterr J Rheumatol
JT  - Mediterranean journal of rheumatology
JID - 101730166
PMC - PMC10466356
OTO - NOTNLM
OT  - IPAF
OT  - classification criteria
OT  - follow-up
COIS- The authors declare no conflict of interest.
EDAT- 2023/09/01 06:43
MHDA- 2023/09/01 06:44
PMCR- 2023/06/30
CRDT- 2023/09/01 03:59
PHST- 2022/07/18 00:00 [received]
PHST- 2023/03/08 00:00 [revised]
PHST- 2023/03/09 00:00 [accepted]
PHST- 2023/09/01 06:44 [medline]
PHST- 2023/09/01 06:43 [pubmed]
PHST- 2023/09/01 03:59 [entrez]
PHST- 2023/06/30 00:00 [pmc-release]
AID - MJR-34-2-180 [pii]
AID - 10.31138/mjr.34.2.180 [doi]
PST - epublish
SO  - Mediterr J Rheumatol. 2023 Jun 30;34(2):180-187. doi: 10.31138/mjr.34.2.180. 
      eCollection 2023 Jun.