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Objectives: To evaluate the rate of progression towards specific autoimmune diseases (SADs) of a prospective, multi-centre cohort of patients classifiable as interstitial pneumonia with autoimmune features (IPAF)., Methods: IPAF patients were enrolled based on specific research criteria, and jointly followed by rheumatologists and pulmonologists for at least one year with clinical check-ups, serological exams including autoimmunity, capillaroscopy and high-resolution computed tomography (HRCT). Diagnostic assessment was repeated at least once a year, or earlier when deemed useful., Results: We enrolled 191 IPAF patients through 95 different combinations of IPAF criteria. Of these, 24.1% progressed towards SAD, mainly in connective tissue diseases but also in microscopic polyangiitis. The IPAF patients who progressed were younger than stable IPAF patients (63±10 years vs. 68±9 years, p=0.002) and had a longer follow-up (36.9±18.7 vs. 29.3±15.7 months, p=0.007), but similar severity. No parameters were associated with overall progression, but some parameters were associated with the development of specific diagnoses: Sjögren's syndrome with positivity for SSA (p=0.007, χ2 7.4); idiopathic inflammatory myopathy with mechanic's hands (p=<0.0001, χ2 12.6), organizing pneumonia pattern (p=0.01, χ2 6.1), positivity for anti-Pm/scl (p=0.04 χ2 4.1) and anti-MDA5 (p=0.04, χ2 4.2); systemic sclerosis with palmar telangiectasias (p=<0.0001 2 18.3), positivity for anti-Scl70 (p=<0.0001 χ2 12.5) and anti-PM/Scl (p=0.001 χ2 10.1)., Conclusions: IPAF patients had a rate of progression towards SAD similar to that reported in previous studies on undifferentiated connective tissue diseases, thus including some patients in which lung involvement could represent the first or even the sole clinical manifestation of a SAD.

Interstitial lung disease (ILD) is one of the most serious pulmonary complications associated with connective tissue diseases (CTDs), resulting in significant morbidity and mortality. Recently, lung ultrasound examination has appeared as a useful, feasible, non-invasive, radiation-free imaging technique with high sensitivity and specificity for the diagnosis of CTD-associated ILD. In this review, we discuss the literature concerning lung ultrasound findings (B-lines and pleural irregularities) in systemic sclerosis. We also examine the possible utility and potential limitations of lung ultrasound in other CTDs including: inflammatory myopathies, mixed connective tissue disease, and Sjögren's syndrome. Specifically, we compare the diagnostic accuracy of lung ultrasound with traditional imaging techniques and pulmonary functional tests.

Objectives: The purpose of this study was to evaluate and compare the diagnostic performance of lung ultrasound (US) in respect to high-resolution computed tomography (HRCT) findings in patients with connective tissue disease-associated interstitial lung disease (CTD-ILD)., Methods: We searched the Pubmed, Embase, and Cochrane Library databases, and performed a meta-analysis on the diagnostic accuracy of lung US according to B-lines (comet tail sign) and on the correlation coefficients between lung US scores and HRCT Warrick scores in CTD-ILD patients., Results: Five studies that included a total of 349 patients were available for this meta-analysis. The pooled sensitivity and specificity of lung US were 91.5% (95% confidence interval [CI]: 84.5-96.0) and 81.3% (95% CI: 74.6-86.9), respectively. The positive likelihood ratio, negative likelihood ratio, and diagnostic odds ratio were 4.100 (2.133-7.879), 0.176 (0.006-0.363), and 34.73 (10.10-99.66), respectively. The area under the curve was 0.915 and the Q* index was 0.848, indicating a high diagnostic accuracy. When all four studies with systemic sclerosis were considered together, the pooled sensitivity and specificity of lung US were 89.5% (95% CI 80.3-95.3) and 79.6% (69.9-87.2), respectively. A significant correlation was found between lung US B-line scores and HRCT Warrick scores in CTD-ILD (correlation coefficient: 0.783; p-value <1 × 10-9)., Conclusions: Our meta-analysis of published studies demonstrates that lung US has a high diagnostic accuracy, correlates well with HRCT findings, and plays an important role in the diagnosis of CTD-ILD.

Rheumatologists have been using ultrasound (US) for the evaluation of patients affected by rheumatic disease for a long time. Actually this approach is becoming more and more diffuse and US is used for multiple purposes: diagnosis, disease activity assessment, prognosis, and therapy monitoring. The real 'new' step for the rheumatologist has been moving from the 'usual' musculoskeletal US to other fields of US, such as the assessment of vascular involvement (both macro and micro), skin, lung and even nails. In this paper we review the published literature related to the use of musculoskeletal, skin and lung US in patients affected by connective tissue diseases.

Ultrasound (US) role is becoming more and more relevant in the assessment of rheumatic diseases but there are still some almost unexplored fields and, surely, one of these is represented by the great family of connective tissue diseases (CTD). In this review we provide an update of the available data regarding some applications of US in CTD. Besides an overview of the role of US in their musculoskeletal involvement, we will report data on the use of US in the evaluation of skin and lung in systemic sclerosis and of salivary glands in Sjögren's syndrome. US assessment of heart, kidney or vascular involvement in CTD will not be the subjects of this paper.