Idiopathic non-specific interstitial pneumonia: as an “autoimmune interstitial pneumonia”

Abstract

Recently, we have experienced significant number of patients diagnosed with non-specific interstitial pneumonia (NSIP) by open lung biopsy or video-assisted thoracoscopic surgery. The purpose of this study is to compare clinical and pathological features of idiopathic NSIP and NSIP associated with underlying diseases (mainly autoimmune disorders). Forty-six patients with histologically proven NSIP were retrospectively collected. Twenty-four patients had underlying diseases (12 polymyositis/dermatomyositis, 5 systemic sclerosis, 2 rheumatoid arthritis, 2 Sjogren's syndrome, 1 ulcerative colitis, 1 primary biliary cirrhosis, and 1 multiple myeloma). Twenty-two of the 46 patients had no underlying diseases. It was very difficult to distinguish idiopathic NSIP and NSIP associated with underlying diseases, clinically and radiologically. Pathologically, Lymphocytic pneumonitis was demonstrated in both groups, and it was impossible to distinguish idiopathic NSIP and NSIP associated with underlying diseases. Since generalized symptoms were not observed in patients with idiopathic NSIP, and clinical and pathological features were identical to NSIP with several autoimmune disorders, we postulate new clinical entities of "autoimmune interstitial pneumonia" in cases without underlying diseases.

Interstitial pneumonia

“…NSIP could be considered identical to CVD-NSIP, because the clinical characteristics were quite similar despite the presence of underlying CVD [23]. Consistent with this, our previous study showed that in terms of prognosis and responsiveness to therapy, idiopathic NSIP was similar to CVD-NSIP [24].…”

The pulmonary histopathologic manifestations of the anti-Jo-1 tRNA synthetase syndrome

“…17,19 Within this group of PM/DM patients, however, is a subgroup with anti-Jo-1 autoantibodies that stand out because of their remarkable association with pulmonary function and chest radiographic abnormalities occurring in up to 90% of cases. 12,[15][16][17]19,21,22 This study represents the largest and most complete study of surgical lung biopsies in this unique patient subset and provides some perspectives on the clinical course of this disease given the inclusion of late stage cases derived from lung transplantation and autopsy programs.…”

“…[1][2][3][4][5][6][7][8][9][10] The anti-Jo-1 tRNA synthetase syndrome has a higher incidence of ILD, relative to classical PM/DM, approximating 90% in some series, and the presence of anti-Jo-1 antibodies correlates with poor patient prognosis. [11][12][13][14][15][16][17][18][19][20][21][22] In this report, we studied the pulmonary histopathologic changes of a select group of IIM patients with the anti-Jo-1 tRNA synthetase syndrome, generating the first and largest biopsy study focused on this specific group of patients.…”

Pivotal clinical dilemmas in collagen vascular diseases associated with interstitial lung involvement

“…Symptoms of RA usually precede lung involvement, but they can occur simultaneously, or RA can follow the diagnosis of IP [8,16,17]. The most frequent respiratory symptoms are progressive dyspnoea and dry cough even if, in many cases, dyspnoea on exercise may be masked by inactivity due to polyarthritis.…”