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Normalization of lung function following treatment of secondary usual interstitial pneumonia: a case report.

Hohberger LA, Montero-Arias F, Roden AC, Vassallo R - Clin Med Insights Circ Respir Pulm Med (2015)

Bottom Line: We present a case of a woman with steroid-responsive biopsy-proven UIP with significant and sustained improvement in pulmonary function.Pulmonary function testing showed moderate impairment with reduction in forced vital capacity (FVC, 69% predicted), forced expiratory volume in one second (FEV1 73% predicted), and diffusing capacity for carbon monoxide (DLCO, 52% predicted).Surgical lung biopsy showed UIP with prominent inflammatory infiltrates.

View Article: PubMed Central - PubMed

Affiliation: Department of Medicine, Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA.

ABSTRACT
Usual interstitial pneumonia (UIP) is the most common idiopathic interstitial pneumonia (IIP) and is associated with a poor prognosis and poor responsiveness to immunosuppressive therapy. We present a case of a woman with steroid-responsive biopsy-proven UIP with significant and sustained improvement in pulmonary function. A female in her 40s presented following a one-year history of progressive dyspnea, a 20 lb weight loss, and fatigue. Imaging of the chest with computed tomography (CT) showed bibasilar subpleural reticular opacities and minimal peripheral honeycombing. Comprehensive connective tissue disease (CTD) antibody testing was negative. Pulmonary function testing showed moderate impairment with reduction in forced vital capacity (FVC, 69% predicted), forced expiratory volume in one second (FEV1 73% predicted), and diffusing capacity for carbon monoxide (DLCO, 52% predicted). Surgical lung biopsy showed UIP with prominent inflammatory infiltrates. Following treatment with prednisone and azathioprine, the patient's symptoms resolved, while objective pulmonary function testing showed normalization of lung function, which is sustained at >4 years of follow-up. Improvement in lung function following immunosuppressive therapy is distinctly uncommon in either idiopathic or secondary UIP. This report suggests that occasionally, patients with secondary UIP occurring in the context of otherwise undefinable autoimmune clinical syndromes may be responsive to immunosuppressive therapy.

No MeSH data available.


Related in: MedlinePlus

Chest radiograph, performed at the time of last follow-up, demonstrating preserved lung volumes and peripheral reticular densities in the mid-lungs and lower lung fields.
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f4-ccrpm-9-2015-023: Chest radiograph, performed at the time of last follow-up, demonstrating preserved lung volumes and peripheral reticular densities in the mid-lungs and lower lung fields.

Mentions: The patient, a female never-smoker in her 40s, presented for evaluation of a one-year history of progressive dyspnea, a 20-pound weight loss, chronic fatigue, arthralgia, and Raynaud’s phenomenon. Prior evaluation had showed an anti-nuclear antibody (ANA) titer of 1:80 and an elevated anti-Sjögren’s-syndrome-related antigen (SSA) antibody. The physical examination was remarkable only for Velcro-type bibasilar crackles. Computed tomography (CT) of the chest showed subpleural reticular opacities most prominent at the bases with minimal areas of early honeycombing (Fig. 1). Comprehensive autoimmune CTD antibody testing was negative (despite known previous ANA positivity). Pulmonary function testing showed proportional reduction in forced vital capacity (FVC), forced expiratory volume in one second (FEV1; 69% and 73% predicted, respectively), and diffusing capacity for carbon monoxide (DLCO; 52% predicted) (Fig. 2). Clinical rheumatology evaluation did not identify a specific CTD. Given the unclear clinical picture, a surgical lung biopsy was performed: in consideration of the chest imaging findings (Fig. 1), wedge biopsies were obtained from the anterior aspect of the right upper lobe and the inferior aspects of the right middle and lower lobes. Surgical lung biopsy showed predominantly mature fibrosis, honeycomb changes with regional heterogeneity, and fibroblast foci consistent with UIP: in addition, there were prominent inflammatory lymphoid infiltrates, mostly involving areas of fibrosis (Fig. 3). In consideration of the patient’s young age, the suspicion of an unclassifiable underlying CTD, and the prominence of lymphoid infiltrates on lung biopsy, a trial of immunosuppression was initiated with prednisone 40 mg/day (0.5 mg/kg) for three months. At her three-month re-evaluation, she reported improved symptoms and significant improvement in spirometry (increase in FVC by 470 mL and FEV1 by 570 mL) as well as DLCO (increase by 12%). Given the response to prednisone, she was initiated on azathioprine and titrated to a dose of 150 mg daily (2 mg/kg). Over the subsequent four years, the prednisone was tapered (dose reduced by 10 mg/day every month while on overlapping azathioprine therapy) and remission of symptoms was maintained with azathioprine. Longitudinal follow-up of lung function testing (performed every four to six months in the first two years after diagnosis and then annually) showed a progressive increase in FVC by a total of 1.1 L (31%). Her FEV1 also improved by 860 mL (31%), and her DLCO by 4.5 (>20%) (Fig. 2). At the time of last follow-up in 2014, the patient was asymptomatic and her pulmonary function test was within normal limits. Nearly five years following initial presentation, the patient underwent full cardiopulmonary exercise testing with graded cycle ergometry and achieved a maximal workload of 175 W and a VO2 max of 90% of that predicted for her age. At the level of highest workload, she breathed at 61% of the maximal voluntary ventilation (indicating normal ventilatory reserve) and recorded a normal ventilatory and gas exchange response throughout exercise. Chest X-ray performed at the most recent follow-up demonstrated normal lung volumes and mild interstitial prominence in the bases (Fig. 4).


Normalization of lung function following treatment of secondary usual interstitial pneumonia: a case report.

Hohberger LA, Montero-Arias F, Roden AC, Vassallo R - Clin Med Insights Circ Respir Pulm Med (2015)

Chest radiograph, performed at the time of last follow-up, demonstrating preserved lung volumes and peripheral reticular densities in the mid-lungs and lower lung fields.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4401236&req=5

f4-ccrpm-9-2015-023: Chest radiograph, performed at the time of last follow-up, demonstrating preserved lung volumes and peripheral reticular densities in the mid-lungs and lower lung fields.
Mentions: The patient, a female never-smoker in her 40s, presented for evaluation of a one-year history of progressive dyspnea, a 20-pound weight loss, chronic fatigue, arthralgia, and Raynaud’s phenomenon. Prior evaluation had showed an anti-nuclear antibody (ANA) titer of 1:80 and an elevated anti-Sjögren’s-syndrome-related antigen (SSA) antibody. The physical examination was remarkable only for Velcro-type bibasilar crackles. Computed tomography (CT) of the chest showed subpleural reticular opacities most prominent at the bases with minimal areas of early honeycombing (Fig. 1). Comprehensive autoimmune CTD antibody testing was negative (despite known previous ANA positivity). Pulmonary function testing showed proportional reduction in forced vital capacity (FVC), forced expiratory volume in one second (FEV1; 69% and 73% predicted, respectively), and diffusing capacity for carbon monoxide (DLCO; 52% predicted) (Fig. 2). Clinical rheumatology evaluation did not identify a specific CTD. Given the unclear clinical picture, a surgical lung biopsy was performed: in consideration of the chest imaging findings (Fig. 1), wedge biopsies were obtained from the anterior aspect of the right upper lobe and the inferior aspects of the right middle and lower lobes. Surgical lung biopsy showed predominantly mature fibrosis, honeycomb changes with regional heterogeneity, and fibroblast foci consistent with UIP: in addition, there were prominent inflammatory lymphoid infiltrates, mostly involving areas of fibrosis (Fig. 3). In consideration of the patient’s young age, the suspicion of an unclassifiable underlying CTD, and the prominence of lymphoid infiltrates on lung biopsy, a trial of immunosuppression was initiated with prednisone 40 mg/day (0.5 mg/kg) for three months. At her three-month re-evaluation, she reported improved symptoms and significant improvement in spirometry (increase in FVC by 470 mL and FEV1 by 570 mL) as well as DLCO (increase by 12%). Given the response to prednisone, she was initiated on azathioprine and titrated to a dose of 150 mg daily (2 mg/kg). Over the subsequent four years, the prednisone was tapered (dose reduced by 10 mg/day every month while on overlapping azathioprine therapy) and remission of symptoms was maintained with azathioprine. Longitudinal follow-up of lung function testing (performed every four to six months in the first two years after diagnosis and then annually) showed a progressive increase in FVC by a total of 1.1 L (31%). Her FEV1 also improved by 860 mL (31%), and her DLCO by 4.5 (>20%) (Fig. 2). At the time of last follow-up in 2014, the patient was asymptomatic and her pulmonary function test was within normal limits. Nearly five years following initial presentation, the patient underwent full cardiopulmonary exercise testing with graded cycle ergometry and achieved a maximal workload of 175 W and a VO2 max of 90% of that predicted for her age. At the level of highest workload, she breathed at 61% of the maximal voluntary ventilation (indicating normal ventilatory reserve) and recorded a normal ventilatory and gas exchange response throughout exercise. Chest X-ray performed at the most recent follow-up demonstrated normal lung volumes and mild interstitial prominence in the bases (Fig. 4).

Bottom Line: We present a case of a woman with steroid-responsive biopsy-proven UIP with significant and sustained improvement in pulmonary function.Pulmonary function testing showed moderate impairment with reduction in forced vital capacity (FVC, 69% predicted), forced expiratory volume in one second (FEV1 73% predicted), and diffusing capacity for carbon monoxide (DLCO, 52% predicted).Surgical lung biopsy showed UIP with prominent inflammatory infiltrates.

View Article: PubMed Central - PubMed

Affiliation: Department of Medicine, Division of Pulmonary and Critical Care Medicine, Mayo Clinic, Rochester, MN, USA.

ABSTRACT
Usual interstitial pneumonia (UIP) is the most common idiopathic interstitial pneumonia (IIP) and is associated with a poor prognosis and poor responsiveness to immunosuppressive therapy. We present a case of a woman with steroid-responsive biopsy-proven UIP with significant and sustained improvement in pulmonary function. A female in her 40s presented following a one-year history of progressive dyspnea, a 20 lb weight loss, and fatigue. Imaging of the chest with computed tomography (CT) showed bibasilar subpleural reticular opacities and minimal peripheral honeycombing. Comprehensive connective tissue disease (CTD) antibody testing was negative. Pulmonary function testing showed moderate impairment with reduction in forced vital capacity (FVC, 69% predicted), forced expiratory volume in one second (FEV1 73% predicted), and diffusing capacity for carbon monoxide (DLCO, 52% predicted). Surgical lung biopsy showed UIP with prominent inflammatory infiltrates. Following treatment with prednisone and azathioprine, the patient's symptoms resolved, while objective pulmonary function testing showed normalization of lung function, which is sustained at >4 years of follow-up. Improvement in lung function following immunosuppressive therapy is distinctly uncommon in either idiopathic or secondary UIP. This report suggests that occasionally, patients with secondary UIP occurring in the context of otherwise undefinable autoimmune clinical syndromes may be responsive to immunosuppressive therapy.

No MeSH data available.


Related in: MedlinePlus