Clinical course and lung function change of idiopathic nonspecific interstitial pneumonia

Abstract

Most studies of idiopathic nonspecific interstitial pneumonia (NSIP) have primarily studied mortality. In order to clarify the detailed outcome and prognostic markers in idiopathic NSIP, the clinical course with initial radiological and clinical features was analysed.The clinical course of 83 patients who were classified with idiopathic NSIP (72 fibrotic, 11 cellular; 27 males and 56 females; mean¡SD age 54.4¡10.1 yrs) was retrospectively analysed.In fibrotic NSIP, 16 (22%) patients died of NSIP-related causes with a median (range) follow-up of 53 (0.3-181) months. Despite the favourable survival (5-yr 74%), patients with fibrotic NSIP were frequently hospitalised with recurrence rate of 36%. Reduced forced vital capacity at 12 months was a predictor of mortality. On follow-up, lung function was improved or stable in ,80% of the patients. The extent of consolidation and ground-glass opacity on initial highresolution computed tomography correlated significantly with serial changes of lung function, and the presence of honeycombing was a predictor of poor prognosis. During follow-up, eight (10%) patients developed collagen vascular disease.In conclusion, the overall prognosis of fibrotic nonspecific interstitial pneumonia was good; however, there were significant recurrences despite initial improvement and a subset of the patients did not respond to therapy. Some patients developed collagen vascular diseases at a later date.

Interstitial lung disease-associated antisynthetase syndrome

“…[6] Although the patient tested negative in this case, the pl-7 and antipl-12 seem to be more frequently associated with the presence of fibrosis in the absence of myositis. [3,4,6] Patients with new-onset and unclear ILD may not display signs of myositis or skin disease in the beginning. The lack of correlation between muscle and pulmonary symptoms often leads to a delayed diagnosis.…”

“…The underlying CT pattern has no influence on disease progression or prognosis. [2,6] The anti-RO52/SSA antibody (a marker for Sjögren syndrome) may occur together with the anti-Jo-1 antibody, as in the case described, and is associated with particularly severe ILD. [7] A combination of drug treatment with a well-structured physiotherapy programme directed at resistance training and inspiratory muscle strength, over a period of several weeks, has an impact on overall wellbeing and symptom improvement.…”

“…[5,8,9] The timely and appropriate treatment with NIV prevented the deterioration during the acute setting. [1,6,8,9] Flow (L/s ) 1 2 We suggest that in the appropriate clinical context, patients with multisystem involvement presenting with arthralgias, Raynaud's phenomenon, myositis and lung involvement should be assessed for the ASS. A negative test for antinuclear antibodies (ANA) does not exclude the ASS syndrome.…”

“…ILD-associated anti-aminoacyl tRNA synthetase (ARS) antibodies are the most prevalent manifestation of ASS. [4][5][6] We report a case of a patient with acute respiratory compromise in the setting of a positive anti-Jo-1 antibody, who was initially treated with non-invasive ventilation (NIV) and corticosteroids (CCSs), immune suppressants and a structured physiotherapy programme.…”

Is It Idiopathic Pulmonary Fibrosis or Not?

“…If progression of disease is seen, treatment with immunosuppressants is thought to be beneficial. 30,31 Steroids are the predominant agent of choice, whereas other immunosuppressants (azathioprine, cyclophosphamide, cyclosporine, and mycophenolate mofetil) are used to supplant steroid therapy. The prognosis for NSIP is generally favorable compared with UIP/IPF, although there is an approximate 20% mortality rate in 5 years.…”