Background: Interstitial lung disease (ILD) includes a group of disorders of the pulmonary parenchyma including ILD secondary to environmental exposure, to sarcoidosis and connective tissue diseases, idiopathic pulmonary fibrosis, nonspecific interstitial pneumonitis. Unclassifiable-ILD (U-ILD) is defined when ILD cannot be included in one of these subtypes, because of inadequate findings or impossibility to complete diagnostic iter. Undifferentiated connective tissue disease (UCTD) is a systemic autoimmune diseases characterized by clinical and serological features typical of other CTD, but not fulfilling any of the existing classification criteria. It has been recently suggested that UCTD should be responsible for ILD, although the available classification criteria do not consider lung manifestations. Differential diagnosis between U-ILD or ILD secondary to UCTD (UCTD-ILD) can be difficult, but fundamental for therapeutic implications. Objectives: To evaluate the clinical and serological features of patients with ILD secondary to UCTD compared to unclassifiable ILD, to improve differential diagnosis and identify patients candidate to immuno-suppressive therapy. Secondary outcome was to construct a clinical algorithm, using a priori variables, helpful to predict ILD-UCTD in clinical practice. Methods: From September 2011 to November 2014, 50 patients referred to our Center were diagnosed for UCTD (26/50) or U-ILD (24/50), after a multidisciplinary discussion according to standard available criteria. Results: Main features and comparison between UCTD-ILD and U-ILD are reported in the table. An inconsistent with usual interstitial pneumonia (UIP) pattern at high resolution computerized tomography (HRCT) was more frequently detected in UCTD-ILD compared to U-ILD. A predictive model based on Raynaud's phenomenon, ocular dryness, and antinuclear antibodies showed a predictive value of 85.7% (UCTD-ILD were correctly classified in 90.5% and U-ILD in 78.6%). Clinical and demographic features of patients with interstitial lung disease UCTD U-ILD p Number of patients 26 24 Sex 19F/7M 14F/10M ns Age at diagnosis 61.7±12.7 67.1±9.1 ns Raynaud's phenomenon 68.0% 29.4% 0.027 Oral dryness 60.0% 35.3% ns Ocular dryness 60.0% 13.3% 0.007 Schirmer test 41.6% 4.2% 0.006 Arthritis 19.2% 0 0.05 Skin manifestations 30.4% 0 0.029 Thrombocytopenia 16.7% 18.2% ns Anemia 33.3% 18.2% ns Antinuclear antibodies 87% 52.4% 0.02 ENA 38.1% 10% ns Rheumatoid factor 13.6% 9.1% ns UIP pattern to HRCT 41.2% 58.8% ns Inconsitent with UIP pattern to HRCT 66.7% 33.3% ns Conclusions: Lung involvement is a possible presenting symptom of UCTD; therefore, differential diagnosis with U-ILD is crucial due to the relevant therapeutic implications; a multidisciplinary approach, including rheumatologist, pulmonologist, radiologist, and pathologist, is mandatory. Some clinical-serological features potentially helpful in differential diagnosis should be carefully evaluated

UNCLASSIFIABLE INTERSTITIAL LUNG DISEASE OR UNDIFFERENTIATED CONNECTIVE TISSUE DISEASE? A CHALLENGING DIFFERENTIAL DIAGNOSIS / Manfredi, Andreina Teresa; Sebastiani, Marco; Cerri, Stefania; DELLA CASA, Giovanni; Giuggioli, D.; Vacchi, Caterina; Colaci, Michele; Spinella, Amelia; Luppi, Fabrizio; Ferri, Clodoveo. - In: ANNALS OF THE RHEUMATIC DISEASES. THE EULAR JOURNAL. - ISSN 1792-328X. - 74:supplement 2(2015), pp. 591-591. ((Intervento presentato al convegno European Congress of Rheumatology tenutosi a Rome nel 10-13 June 2015 [10.1136/annrheumdis-2015-eular.6224].

UNCLASSIFIABLE INTERSTITIAL LUNG DISEASE OR UNDIFFERENTIATED CONNECTIVE TISSUE DISEASE? A CHALLENGING DIFFERENTIAL DIAGNOSIS

MANFREDI, Andreina Teresa;SEBASTIANI, Marco;CERRI, Stefania;DELLA CASA, GIOVANNI;Giuggioli, D.;VACCHI, CATERINA;COLACI, Michele;SPINELLA, AMELIA;LUPPI, Fabrizio;FERRI, Clodoveo
2015-01-01

Abstract

Background: Interstitial lung disease (ILD) includes a group of disorders of the pulmonary parenchyma including ILD secondary to environmental exposure, to sarcoidosis and connective tissue diseases, idiopathic pulmonary fibrosis, nonspecific interstitial pneumonitis. Unclassifiable-ILD (U-ILD) is defined when ILD cannot be included in one of these subtypes, because of inadequate findings or impossibility to complete diagnostic iter. Undifferentiated connective tissue disease (UCTD) is a systemic autoimmune diseases characterized by clinical and serological features typical of other CTD, but not fulfilling any of the existing classification criteria. It has been recently suggested that UCTD should be responsible for ILD, although the available classification criteria do not consider lung manifestations. Differential diagnosis between U-ILD or ILD secondary to UCTD (UCTD-ILD) can be difficult, but fundamental for therapeutic implications. Objectives: To evaluate the clinical and serological features of patients with ILD secondary to UCTD compared to unclassifiable ILD, to improve differential diagnosis and identify patients candidate to immuno-suppressive therapy. Secondary outcome was to construct a clinical algorithm, using a priori variables, helpful to predict ILD-UCTD in clinical practice. Methods: From September 2011 to November 2014, 50 patients referred to our Center were diagnosed for UCTD (26/50) or U-ILD (24/50), after a multidisciplinary discussion according to standard available criteria. Results: Main features and comparison between UCTD-ILD and U-ILD are reported in the table. An inconsistent with usual interstitial pneumonia (UIP) pattern at high resolution computerized tomography (HRCT) was more frequently detected in UCTD-ILD compared to U-ILD. A predictive model based on Raynaud's phenomenon, ocular dryness, and antinuclear antibodies showed a predictive value of 85.7% (UCTD-ILD were correctly classified in 90.5% and U-ILD in 78.6%). Clinical and demographic features of patients with interstitial lung disease UCTD U-ILD p Number of patients 26 24 Sex 19F/7M 14F/10M ns Age at diagnosis 61.7±12.7 67.1±9.1 ns Raynaud's phenomenon 68.0% 29.4% 0.027 Oral dryness 60.0% 35.3% ns Ocular dryness 60.0% 13.3% 0.007 Schirmer test 41.6% 4.2% 0.006 Arthritis 19.2% 0 0.05 Skin manifestations 30.4% 0 0.029 Thrombocytopenia 16.7% 18.2% ns Anemia 33.3% 18.2% ns Antinuclear antibodies 87% 52.4% 0.02 ENA 38.1% 10% ns Rheumatoid factor 13.6% 9.1% ns UIP pattern to HRCT 41.2% 58.8% ns Inconsitent with UIP pattern to HRCT 66.7% 33.3% ns Conclusions: Lung involvement is a possible presenting symptom of UCTD; therefore, differential diagnosis with U-ILD is crucial due to the relevant therapeutic implications; a multidisciplinary approach, including rheumatologist, pulmonologist, radiologist, and pathologist, is mandatory. Some clinical-serological features potentially helpful in differential diagnosis should be carefully evaluated
74
591
591
Manfredi, Andreina Teresa; Sebastiani, Marco; Cerri, Stefania; DELLA CASA, Giovanni; Giuggioli, D.; Vacchi, Caterina; Colaci, Michele; Spinella, Amelia; Luppi, Fabrizio; Ferri, Clodoveo
UNCLASSIFIABLE INTERSTITIAL LUNG DISEASE OR UNDIFFERENTIATED CONNECTIVE TISSUE DISEASE? A CHALLENGING DIFFERENTIAL DIAGNOSIS / Manfredi, Andreina Teresa; Sebastiani, Marco; Cerri, Stefania; DELLA CASA, Giovanni; Giuggioli, D.; Vacchi, Caterina; Colaci, Michele; Spinella, Amelia; Luppi, Fabrizio; Ferri, Clodoveo. - In: ANNALS OF THE RHEUMATIC DISEASES. THE EULAR JOURNAL. - ISSN 1792-328X. - 74:supplement 2(2015), pp. 591-591. ((Intervento presentato al convegno European Congress of Rheumatology tenutosi a Rome nel 10-13 June 2015 [10.1136/annrheumdis-2015-eular.6224].
File in questo prodotto:
File Dimensione Formato  
FRI0451.pdf

Open access

Descrizione: Abstract
Tipologia: Versione pubblicata dall'editore
Dimensione 20.61 kB
Formato Adobe PDF
20.61 kB Adobe PDF Visualizza/Apri
Pubblicazioni consigliate

Licenza Creative Commons
I metadati presenti in IRIS UNIMORE sono rilasciati con licenza Creative Commons CC0 1.0 Universal, mentre i file delle pubblicazioni sono rilasciati con licenza Attribuzione 4.0 Internazionale (CC BY 4.0), salvo diversa indicazione.
In caso di violazione di copyright, contattare Supporto Iris

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11380/1143366
Citazioni
  • ???jsp.display-item.citation.pmc??? ND
  • Scopus ND
  • ???jsp.display-item.citation.isi??? 2
social impact