Progression and prognosis of interstitial pneumonia with autoimmune features: a longitudinal, prospective, multi-centre study

Abstract

ObjectiveTo 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, capillaroscopyand high-resolution computed tomography (HRCT). Diagnostic assessment was repeated at least once a year, or earlier when deemed useful. ResultsWe 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, χ 2 7.4); idiopathic inflammatory myopathy with mechanic's hands (p=<0.0001, χ 2 12.6), organizing pneumonia pattern (p=0.01, χ 2 6.1), positivity for anti-Pm/scl (p=0.04 χ 2 4.1) and anti-MDA5 (p=0.04, χ 2 4.2); systemic sclerosis with palmar telangiectasias (p=<0.0001 2 18.3), positivity for anti-Scl70 (p=<0.0001 χ 2 12.5) and anti-PM/Scl (p=0.001 χ 2 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.

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

“…This proportion is significantly lower than that observed in PU, in which 35.8% of patients were previously classified with other conditions (IPAF or IPF). In the literature, the progression from IPAF to definite CTD is reported in about 24% of cases [22]; however, the current definition of IPAF allows the inclusion of UIP patients only in the presence of at least one item from both the clinical and serological domains [23]. The majority of IPAF patients reported in prospective studies present the minimum of two items (including the morphological domain) for enrolment, and these data explain the limited number of UIP patients classifiable as IPAF [24].…”

Clinical and Serological Characteristics of a Monocentric Cohort of Patients Affected by Interstitial Pneumonia with Autoimmune Features (IPAF)

“…Moreover, interstitial lung disease may be the initial manifestation of a CTD, and it is therefore possible for patients IPAF, to manifest a defined CTD at a later timepoint. 28 , 29 …”

Towards Early Diagnosis of Mixed Connective Tissue Disease: Updated Perspectives

“…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.…”