Dual biologics for severe asthma and atopic dermatitis: Synopsis of two cases and literature review.

The efficacy and safety of the combination of biologic therapies remain unclear with an ineffective and insufficient single biologic for managing asthma. Herein, we report two cases using dual biologics for severe asthma and atopic dermatitis. A 52-year-old male patient who received dupilumab and mepolizumab, benralizumab, or tezepelumab, followed by bronchial thermoplasty, and a 41-year-old male patient who received dupilumab and omalizumab, both experienced improved asthma and atopic dermatitis. To date, 38 cases are using dual biologics for severe asthma. The success rate was 84%, with no major adverse effects. We report the first case of severe asthma receiving dual biologics with tezepelumab and furthermore bronchial thermoplasty, and comprehensive literature review on dual biologics. Dual biologics may be an effective treatment method for severe asthma, requiring further investigation.

Impact of adding pethidine on disinhibition during bronchoscopy with midazolam: a propensity score matching analysis.

BACKGROUND: We sometimes experience disinhibition during bronchoscopy with sedation. However, the impact of adding pethidine on disinhibition has not yet been investigated. This study aimed to examine the additive impact of pethidine on disinhibition during bronchoscopy with midazolam. METHODS: This retrospective study involved consecutive patients who underwent bronchoscopy between November 2019 and December 2020 (sedated with midazolam: Midazolam group) and between December 2020 and December 2021 (sedated with midazolam plus pethidine: Combination group). The severity of disinhibition was defined as follows: moderate, disinhibition that always needed restraints by assistants; and severe, disinhibition that needed antagonization of sedation by flumazenil to continue bronchoscopy. One-to-one propensity score matching was used to match baseline characteristics between both groups. RESULTS: After propensity score matching with depression, the type of bronchoscopic procedure, and the dose of midazolam, 142 patients matched in each group. The prevalence of moderate-to-severe disinhibition significantly decreased from 16.2% to 7.8% (P = 0.028) in the Combination group. The Combination group had significantly better scores for sensation after bronchoscopy and feelings toward bronchoscopy duration than did the Midazolam group. Although the minimum SpO2 during bronchoscopy was significantly lower (88.0 ± 6.2 mmHg vs. 86.7 ± 5.0 mmHg, P = 0.047) and the percentage of oxygen supplementation significantly increased (71.1% vs. 86.6%, P = 0.001) in the Combination group, no fatal complications were observed. CONCLUSIONS: Adding pethidine could reduce disinhibition occurrence in patients undergoing bronchoscopy with midazolam, with better subjective patient outcomes during and after bronchoscopy. However, whether more patients may need oxygen supplementation and whether hypoxia occurs during bronchoscopy should be considered. CLINICAL TRIAL REGISTRATION: UMIN000042635.

One-day systemic corticosteroid administration for asthma and future "short bursts" risk in real clinical practice.

OBJECTIVE: Systemic corticosteroid administration, also called short bursts (SB), is harmful for patients with asthma; however, the actual burden of one-day SB remains unsolved. This study aimed to elucidate the characteristics of patients requiring one-day SB against asthma in clinical practice. METHODS: Consecutive patients who regularly visited our hospital for asthma treatment between January 2019 and December 2020 were reviewed and followed for one year. SB was defined as ≥3 days of systemic corticosteroid treatment for an exacerbation. One-day SB was defined as one-day of systemic corticosteroid to treat an exacerbation. The one-day SB group included patients who received only one-day SB but no SB during the preceding year. Frequent SB was defined as that occurring ≥2 times/year. RESULTS: Data on 229 patients were analyzed. Among them, 2.6% (95% confidence interval 1.2-5.6%) were in the one-day SB group. The one-day SB group was female-dominant, obese, non-eosinophilic, and non-atopic. The median one-day SB was 1.5 times/year and almost half of one-day SB were performed by patients themselves. Independent of the low pulmonary function, high blood eosinophil count, and inhaled corticosteroid dose, one-day SB was associated with future frequent SB (adjusted odds ratio = 18.2, 95% confidence interval 1.1-288, P = 0.040, compared to the no SB group). CONCLUSIONS: Although one-day SB was not frequently experienced, even one-day SB without conventional SB was associated with future frequent SB. It is important to grasp the actual condition of one-day SB and to reinforce the treatment used.

Allergy to Omalizumab: Lessons from a Reaction to the Coronavirus 2019 Vaccine.

Omalizumab can cause hypersensitivity reactions. We herein report the first case of an 18-year-old woman with refractory cough-predominant asthma that correlated with allergic reactions caused by omalizumab and the coronavirus disease 2019 (COVID-19) vaccine. The patient developed angioedema after taking omalizumab. She had previously experienced intense coughing immediately after receiving a COVID-19 vaccine. A skin prick test was positive for polysorbate 20, which was probably the cause of the allergic reactions to omalizumab and the COVID-19 vaccine. Clinicians should check for an allergic reaction, irrespective of its intensity, triggered by polysorbate and be careful when prescribing biologics to patients in order to avoid allergic reactions.

Severe eosinophilia and subsequent dermatologic immune-related adverse event with squamous cell carcinoma antigen elevation induced by nivolumab and ipilimumab.

Immune checkpoint inhibitors (ICIs) for malignant lesions are associated with immune-related adverse events (irAEs), but reports about severe eosinophilia induced by ICIs are scarce. A 73-year-old man with lung squamous cell carcinoma was treated by chemotherapy (carboplatin plus paclitaxel) and ICIs (nivolumab plus ipilimumab). After two cycles of chemotherapy, the ICIs were continued. After 5 months, the eosinophilia, which had exceeded 5000/µl, increasingly deteriorated, and the only detected irAE was a grade 1 rash. Under continuation of the ICIs, although the eosinophilia decreased, a grade 3 rash and severe pruritis subsequently appeared. Squamous cell carcinoma antigen (SCCA) was steeply increased simultaneously. A complete response had been achieved, and oral prednisolone markedly improved the rash, pruritis, and eosinophilia. Clinicians should be aware that precedent severe eosinophilia and subsequent severe irAE could occur in patients treated by nivolumab and ipilimumab, and SCCA elevation could be associated with dermatologic irAE.

Characteristics of patients with frequent systemic corticosteroid bursts for asthma in real clinical practice: are there frequent "short bursts?"

OBJECTIVE: The data on intermittent systemic corticosteroid therapy for asthma exacerbation, clinically called a "short burst," is limited. This study aimed to investigate the characteristics of patients with frequent systemic corticosteroid bursts for asthma in real clinical practice. METHODS: Consecutive patients who regularly visited our hospital for asthma treatment between January 2019 and December 2020 were reviewed. The number of systemic corticosteroid bursts during the past 1 year was collected, and those with frequent bursts (≥2 times/year) were defined as the Frequent group. RESULTS: Data on 236 patients were analyzed. Among them, 5.5% (95% confidence interval 3.2-9.2%) were in the Frequent group. In the Frequent group, 23% of patients had no unplanned visits, and 38% experienced at least one corticosteroid burst without visiting a physician (self-medication). One-third of patients did not undertake high-dose inhaled corticosteroid treatment, and three-fourths of patients did not undertake long-acting muscarinic antagonist treatment. Low pulmonary function and increased blood eosinophils were independently associated with the Frequent group (adjusted odds ratio = 0.73, 95% confidence interval 0.55-0.99, P = 0.039, per 10% predicted increase in a forced expiratory volume in 1 s; adjusted odds ratio = 1.15, 95% confidence interval 1.02-1.29, P = 0.025, per 100/µL increase in blood eosinophils). CONCLUSIONS: There was a certain rate of frequent corticosteroid bursts in real clinical practice. It is important to determine the actual condition, as some patients experienced "hidden" frequent bursts and have the option to reinforce the treatment.

Prevalence and characteristics of disinhibition during bronchoscopy with midazolam.

BACKGROUND: Disinhibition is sometimes experienced during bronchoscopy with sedation. However, data on disinhibition during bronchoscopy are scarce. We examined the prevalence and characteristics of disinhibition during bronchoscopy with midazolam. METHODS: This retrospective study analyzed consecutive patients who underwent bronchoscopy between November 2019 and December 2020. The severity of disinhibition was defined as follows: mild, disinhibition sometimes requiring restraints by assistants; moderate, disinhibition always requiring restraints by assistants; and severe, disinhibition requiring antagonization of sedation by flumazenil to continue bronchoscopy. RESULTS: Among 251 eligible patients who were sedated using midazolam, 36 (14.3%; 95% confidence interval [CI], 10.5%-19.2%), 42 (16.7%; 95% CI, 12.6%-21.8%), and 7 (2.8%; 95% CI, 1.4%-5.6%) experienced mild, moderate, and severe disinhibition, respectively. Depression (odds ratio [OR] 2.77; 95% CI, 1.20-6.41), endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) (OR 10.23; 95% CI, 1.02-103.01, referred to brushing/bronchial washing/observation), and increased administration of midazolam (OR 1.20; 95% CI, 1.02-1.42, per 1-mg increase) were independently associated with moderate-to-severe disinhibition. Patients experiencing moderate disinhibition reported significantly better scores for discomfort during bronchoscopy. Besides the maximum systolic and diastolic blood pressures during bronchoscopy, the changes in hemodynamic and respiratory statuses during bronchoscopy or complications did not significantly differ between patients experiencing moderate-to-severe disinhibition and those experiencing none-to-mild disinhibition. CONCLUSIONS: Moderate-to-severe disinhibition occurred in 19.5% of patients during bronchoscopy with midazolam. We should focus on disinhibition when patients have depression or are planning to undergo EBUS-TBNA, and sparing the administration of midazolam might reduce the occurrence of disinhibition. CLINICAL TRIAL REGISTRATION: UMIN000038571.

Drastically Progressive Ethambutol-induced Optic Neuropathy after Withdrawal of Ethambutol: A Case Report and Literature Review.

Ethambutol-induced optic neuropathy (EON) is a well-known complication, although low-dose ethambutol seldom causes EON. An 85-year-old man with non-tuberculous mycobacterial lung disease was taking antibiotics, including low-dose ethambutol. On day 85 of treatment, the diagnosis of EON was made. Despite prior discontinuation, his best corrected visual acuity drastically deteriorated from 20/17 (right eye) and 20/20 (left eye) to 20/330 (right eye) and 20/1,000 (left eye) within 3 weeks, and this symptom did not resolve. To our knowledge, there have been no reported cases with drastically progressing and irreversible EON even after the withdrawal of low-dose and short-term ethambutol.

Ghost chest tube after talc pleurodesis.

After the talc pleurodesis, CT showed the tract made from the chest tube even after its removal. The unexpanded thoracic space might also contribute to it; thus, gathering specific medical history is important to understand this rare phenomenon.

Ground-glass opacity as a paradoxical reaction in miliary tuberculosis: A case report and review of the literature.

A paradoxical reaction (PR) is an excessive immune response occurring during antitubercular therapy (ATT), but is rare in patients with miliary tuberculosis. A 78-year-old woman complained of general malaise, loss of appetite, and fever for 10 days. Chest computed tomography (CT) showed diffuse, bilateral, discrete miliary nodules. The patient was treated with ATT for miliary tuberculosis. Nine days after starting the treatment, she developed a spiking fever and worsening malaise. Repeat CT showed new localized ground-glass opacity (GGO) in the right upper lobe. After excluding possible etiologies, she was diagnosed with PR due to ATT. She was successfully managed with oral prednisolone while continuing ATT. The GGO diminished and did not recur after discontinuation of the steroids. We reviewed 28 reported cases of miliary tuberculosis with a PR in patients not infected with human immunodeficiency virus. Those not on immunosuppressive therapy were likely to develop a PR early. This case illustrates that a PR may present as localized GGO in miliary tuberculosis in the lung of patients treated with ATT. In cases of a PR with marked symptoms, steroid therapy may be valuable.

Drastically progressive lung cavity lesion caused by Actinomyces odontolyticus in a patient undergoing chemoradiotherapy: A case report and literature review.

Pulmonary actinomycosis reportedly forms 15% of all cases of actinomycosis, and pulmonary Actinomyces odontolyticus is particularly rare. A 60-year-old man with a hoarse voice was referred to our hospital. Lung squamous cell carcinoma was diagnosed at the clinical tumor-node-metastasis stage of cT2N2M0, and concurrent chemoradiotherapy was initiated. Further, a small cavity was also detected in the left upper lobe, but it was observed. During chemoradiotherapy, the small cavity lesion rapidly increased accompanying infiltration, and administration of short-term antibiotics did not improve the patient's condition. Bronchoscopy did not show any diagnostic results. Although a rapidly progressive malignant lesion could not be excluded and surgical management was considered, resection could not be performed because of the tight adhesion of the mass. Therefore, bronchoscopy was performed again, and the bronchial lavage culture showed a positive smear for the Actinomyces species. Further, using matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF MS), the bacteria was identified as A. odontolyticus. After long-term administration of amoxicillin, the lung cavity with infiltration gradually improved. To the best of our knowledge, there have been nine cases of pulmonary A. odontolyticus (excluding those with only empyema or pleural mass without lung lesions), which can occur in immunocompetent patients with persistent lung shadow. None of the cases showed drastic deterioration; therefore, the present case is the first to highlight that A. odontolyticus possibly produce drastically progressive lung cavity lesion. Further, repeated bronchoscopy and MALDI-TOF MS could help to diagnose pulmonary actinomycosis.

Can the Sarcoidosis Health Questionnaire predict the long-term outcomes in Japanese sarcoidosis patients?

RATIONALE: The Sarcoidosis Health Questionnaire (SHQ) is the first sarcoidosis-specific health status questionnaire ever developed. Worse health status, as evaluated by the SHQ, may indicate higher risk for deterioration in the following 5 years. OBJECTIVES: To evaluate the association between SHQ scores and deterioration defined clinically at 5-year follow-up. METHODS: 122 patients with biopsy-supported sarcoidosis completed the SHQ and underwent evaluation with respect to organ involvement, chest radiograph, electrocardiogram, serum biomarker measurements, pulmonary function tests, and echocardiogram. Of these 122, 88 (72.1%) were available for pulmonary, cardiac, and non-pulmonary, non-cardiac deterioration assessment during the following 5 years. MEASUREMENTS AND MAIN RESULTS: Five-year deterioration was observed in 20 patients (23%). The SHQ total score was significantly associated with 5-year deterioration, after adjusting for cardiac involvement at baseline, with adjusted odds ratio (OR) of 0.54 (95% confidence interval [95% CI], 0.29-0.99). The association of the total SHQ with 5-year outcome was not significant when adjusted for left ventricular ejection fraction (LVEF) at baseline (adjusted OR, 0.61 [0.32-1.16]), whereas LVEF was significantly associated with 5-year outcome (adjusted OR, 0.92 [0.86-0.99]). The association between total SHQ score and 5-year deterioration was marginal when adjusted for baseline usage of systemic corticosteroid (CS)/immunosuppressive (IS) agents (adjusted OR, 0.58 [0.31-1.10]), whereas systemic CS/IS usage significantly predicted 5-year deterioration (adjusted odds ratio [OR], 3.46 [1.12-10.7]). There was a marginal correlation between the total SHQ and LVEF (rho = 0.19, p = 0.07) and a weak association between the total SHQ and systemic CS/IS usage (rho = -0.23, p = 0.03). The Physical Functioning domain scores of the SHQ were significantly associated with 5-year deterioration (adjusted OR, 0.45-0.51). CONCLUSIONS: Worse health status, as assessed by the SHQ score, can be a risk factor for 5-year deterioration of sarcoidosis, although usage of the CS/IS at baseline and lower LVEF at baseline are more predictive of 5-year deterioration.

Asymmetry in acute exacerbation of idiopathic pulmonary fibrosis.

Acute exacerbation (AE) of idiopathic pulmonary fibrosis (IPF) results in poor survival. The objective of the present study was to elucidate the impact of asymmetrical ground-glass opacity (GGO) and/or consolidation on outcomes in patients with AE-IPF. The cases of 59 consecutive patients with AE-IPF were retrospectively reviewed. High-resolution computed tomography (HRCT) at diagnosis of an AE was assessed to determine the disease extent and asymmetry. Asymmetrical AE was defined as a right-to-left ratio of GGO and consolidation ≥2.0 or ≤0.5. The impacts of HRCT indices and other clinical parameters on 180-day mortality were analysed. The overall 180-day mortality rate was 59.2%, and asymmetrical AE was observed in 13 patients (22.0%). A multivariate analysis revealed that asymmetrical AE was a significant predictor of 180-day mortality (hazard ratio=0.36, p=0.047), long-term oxygen therapy before AE and serum lactate dehydrogenase levels. The 180-day mortality of patients with asymmetrical AE was significantly lower than that of patients with symmetrical AE (asymmetrical AE 30.8% versus symmetrical AE 68.2%, p=0.03). An asymmetrical distribution of GGO and/or consolidation is a predictor of survival in patients with AE-IPF.

Chronic Kidney Disease Predicts Survival in Patients with Idiopathic Pulmonary Fibrosis.

BACKGROUND: The prevalence of chronic kidney disease (CKD) increases with age as with idiopathic pulmonary fibrosis (IPF). OBJECTIVES: We assessed the prevalence of CKD (stages 3-5) and investigated the relationship of CKD to clinical features and outcomes in patients with IPF. METHODS: This study comprised 123 patients with IPF; 61 subjects with chronic obstructive pulmonary disease (COPD), which was reportedly associated with CKD, were also enrolled as a disease control. CKD (stages 3-5) was defined as an estimated glomerular filtration rate (eGFR) <60 mL/min/1.73 m2. RESULTS: Thirty-seven patients (30%) with IPF and 14 controls (23%) with COPD were diagnosed with CKD, and these frequencies were not significantly different. The patients with IPF and CKD were older (p < 0.01) and had a higher frequency of hypertension (p = 0.048) and ischemic heart disease (p = 0.02) than those with IPF but without CKD. Furthermore, the diffusing capacity of the lung for carbon monoxide (DLCO) and the 6-min walking distance in the patients with CKD were significantly lower (40.0 ± 13.2 vs. 45.9 ± 14.4%, p = 0.04, and 416 ± 129 vs. 474 ± 84 m, p = 0.01, respectively) than in the patients without CKD. The outcome of the patients with CKD showed significantly worse survival compared with the patients without CKD (p = 0.04). Moreover, eGFR remained an independent predictor of survival after adjusting for age and pulmonary function data. CONCLUSION: A substantial percentage of IPF patients have CKD. CKD with a low eGFR was associated with decreased survival in IPF.

The long-term outcome of interstitial lung disease with anti-aminoacyl-tRNA synthetase antibodies.

RATIONALE: Anti-aminoacyl transfer RNA synthetase antibodies (anti-ARS) are a group of myositis-specific autoantibodies that are detected in the sera of patients with polymyositis and dermatomyositis (PM/DM) and also in those of patients with idiopathic interstitial pneumonias without any connective tissue disease (CTD), including PM/DM. Although we reported the clinical characteristics of interstitial lung disease with anti-ARS antibodies (ARS-ILD) with and without PM/DM, the long-term prognosis of ARS-ILD remains undetermined. As our previous studies revealed that ARS-ILD without PM/DM was similar to CTD-associated ILD, and that ARS-ILD with PM/DM was radiologically suggestive of a nonspecific interstitial pneumonia (NSIP) pathological pattern, we hypothesized that the prognosis of ARS-ILD might be distinct from that of idiopathic pulmonary fibrosis (IPF) without anti-ARS. OBJECTIVES: To elucidate the long-term outcome of ARS-ILD with and without PM/DM and compare it to that of IPF. METHODS: A two-center retrospective study was conducted. The study population comprised 36 patients with ARS-ILD (8 with PM, 12 with DM, and 16 without myositis throughout the course), 100 patients with IPF without anti-ARS, and 7 patients with NSIP without anti-ARS. The presence of anti-ARS was determined by RNA immunoprecipitation using the sera obtained at the time of diagnosis before specific treatment. MEASUREMENTS AND MAIN RESULTS: During the observational period (median 49 months; range, 1-114 months), 7 patients with ARS-ILD (19%; 3 with PM, 1 with DM, and 3 without PM/DM) and 51 patients with IPF (51%) died. Patients with ARS-ILD had better overall survival than those with IPF (log-rank test, P < 0.001) and similar survival compared to those with NSIP (log-rank test, P = 0.59). The prognosis for patients with ARS-ILD was similar between those with and without myositis (log-rank test, P = 0.91). At the median follow-up time of 76.5 months, 14 of the 36 patients with ARS-ILD had deteriorated. Both a decline in forced vital capacity or an initiation of long-term oxygen therapy during the course (odds ratio [OR], 5.34) and acute exacerbation (OR, 28.4) significantly increased the mortality risk. CONCLUSIONS: The long-term outcome of ARS-ILD was significantly better than that of IPF regardless of the presence or absence of myositis.

Comprehensive evaluation of airway involvement in pulmonary sarcoidosis.

IOS can predict airway hyperresponsiveness and long-term outcome in patients with pulmonary sarcoidosis http://ow.ly/bkQH307VD4m.

Platelet aggregability in patients with interstitial pneumonias.

BACKGROUND: Recent epidemiological studies have shown that patients with interstitial pneumonia have an increased risk of cardiovascular events. Although the presence of a coagulation/fibrinolysis abnormality in idiopathic pulmonary fibrosis (IPF) has been reported, platelet aggregability has not been evaluated in interstitial pneumonias. This study aimed to investigate platelet aggregability in patients with interstitial pneumonias. METHODS: This observational cohort study included 59 patients with interstitial pneumonias [19 with IPF, 23 with other idiopathic interstitial pneumonias (IIPs), and 17 with connective tissue disease-associated interstitial pneumonias (CTD-IPs)] and 23 healthy control subjects. ADP- and collagen-induced platelet aggregability was measured together with coagulation/fibrinolysis markers. Whole blood (WB) and platelet rich plasma platelet aggregation were measured using the screen filtration pressure and optical aggregometer techniques, respectively. The platelet aggregation threshold index (PATI) was calculated; a lower PATI indicated enhanced platelet aggregability. RESULTS: ADP-induced WB-PATI was significantly decreased in CTD-IPs [log WB-PATI median 0.31 (inter-quartile range, 0.07-0.34) µM, n = 17] compared with that in controls [0.35 (0.32-0.45) µM, n = 23] (p < 0.05). However, there was no significant difference in platelet aggregability between the other patient groups and controls. In contrast, d-dimer, thrombin-antithrombin complex, and von Willebrand factor levels were significantly higher in all patient groups compared with those in controls (p < 0.001). Platelet aggregability was not associated with either disease severity or survival. CONCLUSIONS: Serum coagulation and fibrinolysis markers significantly increased in IIPs and CTD-IPs. In contrast, platelet aggregability was only weakly enhanced in CTDs, but not in IIPs.

Clinical impact of high-attenuation and cystic areas on computed tomography in fibrotic idiopathic interstitial pneumonias.

BACKGROUND: Quantitative computed tomography (CT) analysis has been proposed as a means of objectively assessing fibrotic interstitial pneumonia (IP) including idiopathic pulmonary fibrosis (IPF). We investigated whether percentages of high-attenuation areas (HAA%) and cystic areas (CA%) quantified from CT images were useful as indices of fibrotic IP. METHODS: CT images of 74 patients with fibrotic idiopathic interstitial pneumonias (IPF, 36; non-specific interstitial pneumonia, 9; unclassifiable idiopathic interstitial pneumonia, 29) were analyzed via in-house computer software, which automatically calculated HAA%, CA%, mean lung density (MLD), standard deviation of lung density (SD-LD), kurtosis, and skewness from CT attenuation histograms. These indices were compared in each instance with physiologic measures, visual fibrosis score, clinical diagnosis, radiologic CT pattern, and prognosis. RESULTS: HAA% correlated significantly with physiologic measures and visual fibrosis score to a moderate extent (%forced vital capacity, rs = -0.59; % carbon monoxide diffusion capacity, rs = -0.43; fibrosis score, rs = 0.23). Densitometric parameters (MLD, SD-LD, kurtosis, and skewness) correlated significantly with physiologic measures and fibrosis score (|rs| = 0.28-0.59). CA% showed no association with pulmonary functions but differed significantly between IPF and other interstitial pneumonias (IPs) (1.50 ± 2.41% vs. 0.41 ± 0.80%; P < 0.01) and between the definite usual interstitial pneumonia (UIP) pattern and other patterns (1.48 ± 2.38% vs. 0.55 ± 1.19%; P < 0.01). On univariate analysis, HAA%, MLD, SD-LD, kurtosis, skewness, fibrosis score, and definite UIP pattern all correlated with survival, with kurtosis alone identified as a significant predictor of mortality on multivariate analysis (hazard ratio = 0.67; 95% CI, 0.44-0.96; P = 0.03). CONCLUSION: CA% and HAA% are novel quantitative CT indices with differing properties in fibrotic IP evaluations. HAA% largely reflects physiologic impairments, whereas CA% corresponds with diagnosis and HRCT pattern. Of the CT indices examined, kurtosis constituted the strongest predictor of mortality.

Bone mineral density in patients with idiopathic pulmonary fibrosis.

BACKGROUND: Decreased bone mineral density (BMD) has been reported in patients with interstitial lung disease. However, BMD has not been evaluated in steroid-naïve patients with idiopathic pulmonary fibrosis (IPF). We aimed to measure vertebral BMD and investigate its relationship with clinical features in steroid-naïve patients with IPF. METHODS: We recruited 55 consecutive male patients with steroid-naïve IPF; 55 male smokers without chronic obstructive pulmonary disease or interstitial lung disease, matched by age, body mass index, and pack-years of smoking (control smokers); and 27 healthy young adults. Thoracic vertebral BMD was measured by computed tomography (CT). We further investigated the relationship of BMD with clinical features and quantitative CT indices of lung density in patients with IPF. RESULTS: The thoracic vertebral BMD of patients with IPF was significantly lower than that of control smokers (139.9 ± 28.5 mg/mL vs 160.9 ± 39.5 mg/mL, p < 0.01). Fifteen patients (27.2%) had BMD more than 2.5 SD below the mean BMD of young adults. In patients with IPF, emphysema volume (EV) and its ratio to total lung volume (EV%) had a significantly negative correlation with BMD (r = -0.28, p = 0.04 and r = -0.39, p < 0.01, respectively). In stepwise multiple regression analysis, EV% was an independent explanatory variable for thoracic vertebral BMD. CONCLUSION: A substantial percentage of steroid-naïve IPF patients had decreased BMD, and a significant association was observed between the extent of emphysema and BMD in IPF.