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Jay H Ryu

Publications and source records attributed to Jay H Ryu.

At least 55 records · Page 3Linked to original sources
PubMed2004

Transforming growth factor-beta 1 and extracellular matrix-associated fibronectin expression in pulmonary lymphangioleiomyomatosis.

STUDY OBJECTIVES: Lymphangioleiomyomatosis (LAM) is a rare disorder of unknown etiology, affecting almost exclusively women of childbearing age, that is associated with the proliferation of spindle cells and cystic changes in the affected lung. The underlying processes that contribute to this disease are poorly understood. Transforming growth factor (TGF)-beta(1) is a potent cytokine that promotes mesenchymal cell proliferation and regulates the synthesis of extracellular matrix (ECM) components, particularly fibronectins. Herein, we evaluate the expression of TGF beta(1) and matrix-associated fibronectin in lung specimens demonstrating LAM. DESIGN: Lung biopsy specimens that were confirmed to contain pathologic LAM cells were obtained from 13 patients. The specimens were submitted to immunohistochemical evaluation for TGF beta(1) and fibronectin, as well as the typical markers of LAM cells. Healthy lung parenchyma surrounding resected neoplasms was studied in a parallel fashion as control tissues. MEASUREMENTS AND RESULTS: In all 13 LAM cases and in healthy lung parenchyma, we demonstrated that TGF beta(1) localized consistently to airway epithelial cells. However, in LAM tissues, matrix-associated TGF beta(1) was also consistently found in regions containing pathologic LAM cells. Notably, more abundant TGF beta(1) was observed in highly cellular areas compared to the walls of chronic cystic regions in LAM tissues. Fibronectin, a matrix component that is strongly expressed in response to active TGF beta(1) was found to consistently colocalize with this protein in these highly cellular regions, supporting TGF beta(1) activity in these regions. The markers of proliferating LAM cells, including proliferating cell nuclear antigen, were also markedly present in these highly cellular LAM regions. CONCLUSION: These studies suggest that the proliferation of aberrant LAM cells may be associated with altered regional expression of TGF beta(1) and related ECM proteins.

Actins↗
PubMed2004

Combined corticosteroid and cyclophosphamide therapy does not alter survival in idiopathic pulmonary fibrosis.

STUDY OBJECTIVES: Treatment of patients with idiopathic pulmonary fibrosis (IPF) conventionally includes corticosteroids and cytotoxic agents. No study to date has adequately evaluated the benefits of this approach. This study retrospectively compared combination corticosteroid and cyclophosphamide therapy in a large population of patients who meet the current consensus definition of IPF. DESIGN: Patients were identified retrospectively and treatment addressed on an intention-to-treat basis. Treated and untreated patients were matched by age and percentage of predicted FVC (FVC%) at the time of the initial visit. SETTING: Two academic tertiary referral centers. PATIENTS OR PARTICIPANTS: The diagnosis of IPF was based on current consensus criteria. A total of 164 patients (82 treated and 82 untreated) were included. INTERVENTIONS: Treatment consisted of combined corticosteroid and cyclophosphamide therapy using a standardized protocol. MEASUREMENTS AND RESULTS: There was no difference in age, FVC%, gender, or smoking status between groups. No survival difference was found between patients who were treated (median survival, 1,431 days) or untreated (median survival, 1,665 days) [p = 0.58]. The lack of treatment effect persisted when only those patients with a diagnosis by surgical biopsy (n = 24) or FVC% >/= 60 (n = 107) were analyzed. CONCLUSIONS: Our data suggest that combined corticosteroid and cyclophosphamide therapy has no impact on survival in patients with IPF. This finding supports the evolving concept that chronic inflammation plays a minimal role in the progression of IPF and reinforces the importance of careful consideration of the risks and benefits of such therapies prior to their institution.

Adrenal Cortex Hormones↗
PubMed2004

Impact of angiotensin-converting enzyme inhibitors and statins on survival in idiopathic pulmonary fibrosis.

STUDY OBJECTIVES: To assess the clinical relevance of angiotensin-converting enzyme inhibitors (ACEI) and 3-hydroxy-3-methylglutaryl coenzyme-A reductase inhibitors (statins) in the context of idiopathic pulmonary fibrosis (IPF). BACKGROUND: IPF is a progressive interstitial lung disease for which there is no effective treatment. ACEI and statins have been shown to possess antifibrotic properties in experimental models in vitro and in vivo. DESIGN, SETTING, AND PATIENTS: Retrospective review of the effects of ACEI and statins on survival of 478 patients with IPF seen at Mayo Clinic Rochester from 1994 through 1996. Fifty-two patients (11%) were receiving ACEI, 35 patients (7%) were receiving statins, and 5 patients (1%) patients were receiving both at their initial visit. RESULTS: For subjects receiving ACEI, the median survival from the index visit was 2.2 years, compared to 2.9 years for subjects not receiving ACEI (p = 0.088). The median survival was 2.9 years if patients were receiving statins or not (p = 0.573). There was also no significant difference in survival between patients with IPF receiving either ACEI or statins vs those receiving neither at the index visit (2.5 years vs 3 years, respectively; p = 0.066). After adjusting for age, gender, recommended IPF treatment, smoking status, prior oxygen use, FVC, diffusion capacity for carbon monoxide, coronary artery disease, congestive heart failure, diabetes mellitus, and hypertension, there were no differences in survival between those subjects receiving either ACEI, statins, or both vs neither. CONCLUSIONS: These data do not suggest a beneficial effect of ACEI and/or statins on survival in patients with IPF.

Adult↗
PubMed2004

Echocardiographic and clinical characteristics of pulmonary hypertension complicating pulmonary Langerhans cell histiocytosis.

OBJECTIVE: To determine the echocardiographic characteristics and impact on survival of pulmonary hypertension (PH) and correlations between echocardiographic and clinical variables in patients with pulmonary Langerhans cell histiocytosis (PLCH). PATIENTS AND METHODS: Of 123 adults seen at our institution between January 1976 and December 2002 with histologically proven PLCH, 17 underwent echocardiographic evaluation. Correlations were performed between echocardiographic measures of PH and clinical variables. Cumulative survival probabilities for patients with PH were estimated using the Kaplan-Meier method and were compared to a historical cohort of patients with PLCH using time-dependent proportional hazard regression. RESULTS: Of the 17 patients, PH (estimated pulmonary artery systolic pressure [PASP] at rest, > 35 mm Hg) was present in 15. Thirteen patients (6 men; median PASP, 67 mm Hg; range, 41.2-90.6 mm Hg) had no other known causes of PH. All patients were smokers. Nine patients had a PASP of more than 50 mm Hg. An inverse correlation was found between the forced vital capacity and PASP (r = -0.61; P = .03); no correlation was found between PASP and other pulmonary function parameters. Seven patients with a PASP greater than 65 mm Hg had an enlarged right ventricle with impaired systolic function. The development of PH in patients with PLCH was associated with increased mortality (hazard ratio, 22.8; 95% confidence interval, 7.6 to > 68.9; P < .001). CONCLUSION: Severe PH occurs in PLCH, correlates with the forced vital capacity, and has a significant impact on survival. Clinicians should consider echocardiographic screening for PH in all dyspneic patients with PLCH.

Adult↗
PubMed2004

Sensitivity and specificity of the semiquantitative latex agglutination D-dimer assay for the diagnosis of acute pulmonary embolism as defined by computed tomographic angiography.

OBJECTIVE: To determine the sensitivity and specificity of the semiquantitative latex agglutination plasma fibrin D-dimer assay for the diagnosis of acute pulmonary embolism by using computed tomographic (CT) angiography as the diagnostic reference standard. PATIENTS AND METHODS: From January 1, 1998, to June 26, 2000, patients who had both semiquantitative latex agglutination plasma fibrin D-dimer testing and CT angiography for suspected acute pulmonary embolism were selected for the study. A D-dimer value greater than 250 ng/mL was considered positive for thromboembolic disease. Diagnosis of acute pulmonary embolism was based solely on the interpretation of the CT angiogram. The D-dimer assay results were then compared with the CT angiographic diagnoses. RESULTS: Of 946 CT studies, 172 (18%) were positive for acute pulmonary embolism. The D-dimer assay was positive for 612 (65%) of the 946 patients. For acute pulmonary embolism, the D-dimer assay had a sensitivity of 0.83 (95% confidence interval [CI], 0.76-0.88), a specificity of 039 (95% CI, 036-0.43), a negative likelihood ratio of 0.44 (95 % CI, 032-0.62), and a negative predictive value of 0.91 (95% CI, 0.87-0.94). CONCLUSIONS: The semiquantitative latex agglutination plasma fibrin D-dimer assay had moderate sensitivity and low specificity for the diagnosis of acute pulmonary embolism. When used alone, the results of this test were insufficient to exclude this serious and potentially fatal disorder. Approximately two thirds of our patients had positive D-dimer assays and required further evaluation to exclude acute pulmonary embolism.

Adolescent↗
PubMed2004

Aspergillus infection of implantable cardioverter-defibrillator.

The use of pacemakers and implantable cardioverter-defibrillators continues to increase for the management of cardiac dysrhythmias and, more recently, heart failure. Long-term complications associated with their use include infection, lead failure, and spurious shocks. Although the risk of infection with intracardiac devices is well known, the clinical presentation of this complication can be insidious, delayed in onset, and difficult to diagnose. We report a case of Aspergillus fumigatus infection of an implantable cardioverter-defibrillator with right-sided endocarditis in a 55-year-old man. The infection presented as persistent pulmonary infiltrates (due to recurrent septic pulmonary embolism) and anemia more than 2 years after implantation of the device. Clinicians should be aware of the variable manifestations resulting from infection of intracardiac devices.

Amphotericin B↗
PubMed2004

Clinicoradiological features of pulmonary infarctions mimicking lung cancer.

OBJECTIVE: To describe presenting features of pulmonary infarction that may simulate those of lung cancer. PATIENTS AND METHODS: We reviewed the medical records of 43 patients with pulmonary infarction diagnosed by surgical lung biopsy at the Mayo Clinic in Rochester, Minn, from January 1, 1996, to December 31, 2002. Of 16 patients presenting with an undiagnosed solitary pulmonary nodule or mass, 6 had features suggestive of lung cancer on additional imaging, including abnormalities on contrast-enhancement computed tomography (CT), positron emission tomography (PET), or nonsurgical lung biopsy before surgical resection. We examined the presenting symptoms, epidemiological, clinical, and radiological features, and clinical course of these 6 patients. RESULTS: All 6 patients, ranging in age from 41 to 85 years, had a history of smoking and underlying cardiopulmonary disease. In 5 of the 6 patients, CT showed a nodule in the subpleural region of the lung. Three patients had abnormalities on contrast-enhancement CT, 2 had abnormalities on PET, and 1 had abnormal cytologic findings on a transthoracic needle biopsy of the lung; all these studies showed abnormalities suggestive of lung cancer. Surgical resection of the nodule or mass revealed pulmonary infarction associated with organizing thrombi in all 6 patients. CONCLUSIONS: Pulmonary infarctions can closely mimic the clinicoradiological characteristics of lung cancer, an association not reported previously. Furthermore, cytologic changes that occur in pulmonary infarctions may produce malignant-appearing cells on needle biopsy of the lung. The possibility of pulmonary infarction should be considered in the differential diagnosis of a solitary lung nodule or mass located in the subpleural region, even in the absence of clinically recognized venous thromboembolism.

Adult↗
PubMed2004

Pulmonary involvement in Henoch-Schönlein purpura.

OBJECTIVE: To describe pulmonary involvement in Henoch-Schönlein purpura (HSP). PATIENTS AND METHODS: We retrospectively reviewed the medical records of patients with HSP who were seen at the Mayo Clinic in Rochester, Minn, during a 6-year period (January 1, 1997, to December 31, 2002). Patients with HSP and pulmonary involvement were identified through a review of clinical records, radiological studies, pulmonary function data, and lung biopsy findings. RESULTS: We identified 124 patients with HSP during the study period; 72 (58.1%) were males. The median age was 15 years (range, 8 months to 81 years). Among the 124 patients with HSP, 3 (2.4%), all adults, had pulmonary involvement. Of these 3 patients, aged 20, 67, and 76 years, 2 were women. Pulmonary manifestations consisted of diffuse alveolar hemorrhage (DAH) (2 patients) and usual interstitial pneumonia that improved with corticosteroid therapy (1 patient). CONCLUSIONS: Pulmonary involvement in HSP is rare. It occurs more often in adults and commonly manifests as DAH and occasionally as usual interstitial pneumonia or interstitial fibrosis. Our cases and previously reported cases suggest that DAH is the most common manifestation of pulmonary involvement in HSP.

Adolescent↗
PubMed2004

Thoracic surgical procedures in patients with rheumatoid arthritis.

OBJECTIVE: Rheumatoid arthritis (RA) is associated with a variety of pleuropulmonary manifestations, some of which require surgical intervention. We investigated the spectrum of indications and results, as well as outcome associated with thoracic surgical procedures in rheumatoid patients. METHODS: Identification and retrospective review of medical records of 100 patients with RA at a tertiary-referral medical center undergoing thoracic surgical procedures over a 24-year period from January 1, 1976, to December 31, 1999. RESULTS: Sixty-four patients underwent surgical lung biopsy for localized lung lesions, 57 of which were nodules or masses; 24 lesions proved to be malignant. Surgical lung biopsy was undertaken in 40 patients for diffuse interstitial lung disease, 4 cases of which were found to be infectious. Five other patients underwent surgery for pleural disease, 3 of which proved to be benign pleuritis. CONCLUSION: Patients with rheumatoid disease undergo thoracic surgical procedures for a variety of indications, including benign and malignant localized lesions and pleural disease as well as diffuse parenchymal lung disease. The overall in-hospital mortality rate was low. However, the subgroup with diffuse interstitial lung disease was found to have a 20% in-hospital death rate following surgical lung biopsy.

Aged↗
PubMed2003

Bronchiolar disorders.

Bronchiolar abnormalities are relatively common and occur in a variety of clinical settings. Various histopathologic patterns of bronchiolar injury have been described and have led to confusing nomenclature with redundant and overlapping terms. Some histopathologic patterns of bronchiolar disease may be relatively unique to a specific clinical context but others are nonspecific with respect to either etiology or pathogenesis. Herein, we present a scheme separating (1) those disorders in which the bronchiolar disease is the predominant abnormality (primary bronchiolar disorders) from (2) parenchymal disorders with prominent bronchiolar involvement and (3) bronchiolar involvement in large airway diseases. Primary bronchiolar disorders include constrictive bronchiolitis (obliterative bronchiolitis, bronchiolitis obliterans), acute bronchiolitis, diffuse panbronchiolitis, respiratory bronchiolitis, mineral dust airway disease, follicular bronchiolitis, and a few other rare variants. Prominent bronchiolar involvement may be seen in several interstitial lung diseases, including hypersensitivity pneumonitis, respiratory bronchiolitis-associated interstitial lung disease, cryptogenic organizing pneumonia (idiopathic bronchiolitis obliterans organizing pneumonia), and pulmonary Langerhans' cell histiocytosis. Large airway diseases that commonly involve bronchioles include bronchiectasis, asthma, and chronic obstructive pulmonary disease. The clinical relevance of a bronchiolar lesion is best determined by identifying the underlying histopathologic pattern and assessing the correlative clinico-physiologic-radiologic context.

Bronchi↗
PubMed2003

Pulmonary cryptococcosis in nonimmunocompromised patients.

BACKGROUND: Cryptococcus neoformans can cause serious systemic infections requiring systemic antifungal therapy in immunocompromised hosts. However, isolated pulmonary cryptococcosis in nonimmunocompromised hosts has been reported to resolve spontaneously without treatment. STUDY OBJECTIVE: s: To determine the role of antifungal therapy in the management of isolated pulmonary cryptococcosis in nonimmunocompromised hosts. DESIGN: Retrospective study. SETTING: Tertiary care, referral medical center PATIENTS: Thirty-six nonimmunocompromised subjects with isolated pulmonary cryptococcosis who received diagnoses at the Mayo Clinic (Rochester, MN) from 1976 to 2001. INTERVENTIONS: None. MEASUREMENTS AND RESULTS: Of 42 nonimmunocompromised subjects with cryptococcal infections, 36 (86%) had isolated pulmonary cryptococcosis. The mean (+/- SD) age of these 36 patients was 61 +/- 15 years (range, 14 to 88 years), and the groups included 17 men (47%) and 19 women (53%). Twenty-four patients (67%) were symptomatic, and 12 patients (33%) were asymptomatic. The most common presenting symptoms were cough, dyspnea, and fever. Cultures of sputum and bronchial washings most commonly yielded the diagnosis. Cerebrospinal fluid examination was performed in 11 patients (31%) and was negative in all of them. Follow-up information was available on 25 patients (69%) with a median duration of 19 months (range, 1 to 330 months). Twenty-three of these patients (92%) had resolution of their disease (no treatment, 8 patients; surgical resection only, 6 patients; and antifungal therapy, 9 patients). The condition of the two remaining patients had improved. There was no documented treatment failure, relapse, dissemination, or death in any of these 25 patients. CONCLUSIONS: Our findings suggest that an initial period of observation without the administration of antifungal therapy is a reasonable option for nonimmunocompromised subjects with pulmonary cryptococcosis in the absence of systemic symptoms or evidence of dissemination, as well as after surgical resection for focal cryptococcal pneumonia.

Adolescent↗
PubMed2003

Chylothorax in lymphangioleiomyomatosis.

STUDY OBJECTIVE: Pulmonary lymphangioleiomyomatosis (LAM) is a rare, serious disorder characterized by proliferation of abnormal smooth-muscle cells and affects almost exclusively women of childbearing age. Optimal management of chylothorax, a well-recognized complication of LAM, in these patients has not been defined. This study was performed to characterize the clinical course and identify appropriate management options for chylothorax occurring in patients with LAM. DESIGN: Identification and retrospective review of available medical records on patients with LAM and chylothorax. SETTING: Tertiary-referral medical center. PATIENTS: All patients with LAM seen at Mayo Clinic, Rochester, MN, from January 1, 1976, to December 31, 2000. INTERVENTION: None. MEASUREMENT AND RESULTS: Eight of 79 patients (10.1%) with LAM had chylothorax. All were women aged 33 to 51 years, and four patients had underlying tuberous sclerosis complex. These eight women represented 3.5% of the 229 patients with chylothorax seen over this 25-year period at Mayo Clinic Rochester. Six patients had unilateral pleural effusion and two patients had bilateral effusions at initial presentation. The size of the chylothorax varied and was not necessarily progressive. Management of chylothorax ranged from thoracentesis only to thoracotomy with thoracic duct ligation and parietal pleurectomy. When needed, pleurodesis by instillation of sclerosing agents or parietal pleurectomy appeared to be effective in controlling chylothorax. CONCLUSIONS: Chylothorax occurring in patients with LAM has a variable clinical course. Although pleurodesis with or without thoracic duct ligation appears to be effective in controlling intractable chylothorax, less invasive treatments such as thoracentesis or observation may suffice in some cases. Management of chylothorax in patients with LAM should be individualized depending on the size and clinical effects of the chylous pleural effusion, as well as comorbid factors and local expertise.

Adult↗
PubMed2003

The overlap between respiratory bronchiolitis and desquamative interstitial pneumonia in pulmonary Langerhans cell histiocytosis: high-resolution CT, histologic, and functional correlations.

OBJECTIVES: To characterize the histologic, high-resolution CT (HRCT), and functional correlates of respiratory bronchiolitis (RB)/desquamative interstitial pneumonia (DIP)-like changes in biopsy specimens of adults with pulmonary Langerhans cell histiocytosis (PLCH). METHODS: We retrospectively identified 14 adult patients with histologically proven PLCH (all smokers) in whom both biopsy specimens and HRCT were available for review. The presence and extent of RB/DIP-like changes on lung biopsy specimens of patients with PLCH were correlated with total pack-years of smoking, lung function variables, and the presence of ground-glass attenuation on HRCT. RESULTS: Varying degrees of RB/DIP-like changes were identified in all biopsy specimens. The extent of involvement with RB/DIP-like changes correlated with the cumulative exposure to cigarettes smoked at the time of biopsy (r = 0.61, p = 0.03). Ground-glass attenuation was detected in three HRCTs, two of which had extensive RB/DIP-like changes (> or = 70% involvement) on histology, suggesting that substantial smoking-induced RB/DIP-like changes may be the cause of ground-glass attenuation on the HRCT of patients with PLCH. No correlation was found between the extent of RB/DIP-like changes and total lung capacity, FEV(1), or diffusion capacity of the lung for carbon monoxide. CONCLUSIONS: RB/DIP-like changes are exceedingly common in PLCH, may be sufficiently severe to cause the appearance of ground-glass attenuation on HRCT, and correlate with the cumulative exposure to cigarettes smoked. This study provides additional evidence that PLCH, RB, and DIP form a spectrum of interstitial patterns of lung injury to cigarette smoke.

Bronchiolitis↗
PubMed2003

Cytomegalovirus pneumonia mimicking lung cancer in an immunocompetent host.

Cytomegalovirus (CMV) pneumonia can be a life-threatening disease in immunocompromised patients such as transplant recipients and patients given immunosuppressive therapy. Although CMV infections are highly prevalent in the general population, symptomatic pneumonia in an immunocompetent adult has been documented rarely. We describe a 47-year-old male smoker who presented with a 3.5-cm cavitary mass in the upper lobe of the left lung, highly suggestive of lung cancer. Wedge resection of the mass on thoracotomy revealed CMV pneumonia with no evidence of malignancy or other infections. No antiviral therapy was given to this immunocompetent patient, and no additional manifestations of CMV disease occurred.

Cytomegalovirus Infections↗
PubMed2003

Cystic and cavitary lung diseases: focal and diffuse.

Cysts and cavities are commonly encountered abnormalities on chest radiography and chest computed tomography. Occasionally, the underlying nature of the lesions can be readily apparent as in bullae associated with emphysema. Other times, cystic and cavitary lung lesions can be a diagnostic challenge. In such circumstances, distinguishing cysts (wall thickness < or = 4 mm) from cavities (wall thickness > 4 mm or a surrounding infiltrate or mass) and focal or multifocal disease from diffuse involvement facilitates the diagnostic process. Other radiological characteristics, including size, inner wall contour, nature of contents, and location, when correlated with the clinical context and tempo of the disease process provide the most helpful diagnostic clues. Focal or multifocal cystic lesions include blebs, bullae, pneumatoceles, congenital cystic lesions, traumatic lesions, and several infectious processes, including coccidioidomycosis, Pneumocystis carinii pneumonia, and hydatid disease. Malignant lesions including metastatic lesions may rarely present as cystic lesions. Focal or multifocal cavitary lesions include neoplasms such as bronchogenic carcinomas and lymphomas, many types of infections or abscesses, immunologic disorders such as Wegener granulomatosis and rheumatoid nodule, pulmonary infarct, septic embolism, progressive massive fibrosis with pneumoconiosis, lymphocytic interstitial pneumonia, localized bronchiectasis, and some congenital lesions. Diffuse involvement with cystic or cavitary lesions may be seen in pulmonary lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis, honeycomb lung associated with advanced fibrosis, diffuse bronchiectasis, and, rarely, metastatic disease. High-resolution computed tomography of the chest frequently helps define morphologic features that may serve as important clues regarding the nature of cystic and cavitary lesions in the lung.

Cysts↗
PubMed2003

Interferon gamma-1b therapy for advanced idiopathic pulmonary fibrosis.

OBJECTIVE: To report on observations made in a group of patients with idiopathic pulmonary fibrosis/usual interstitial pneumonia (IPF/UIP) who were treated with interferon gamma-1b. PATIENTS AND METHODS: We reviewed the clinical records, radiological studies, and pulmonary function data of all patients treated with interferon gamma-1b between January 2000 and March 2002 at the Mayo Clinic in Rochester, Minn. RESULTS: Twenty-one patients (mean age, 68 years; range, 52-80 years) were treated with interferon gamma-1b for a mean duration of 82 months (range, 3-21 months). All patients had been diagnosed previously as having IPF/UIP based on clinical, pulmonary function, and chest high-resolution computed tomographic scan criteria; 12 patients had also undergone video-assisted thoracoscopic surgical lung biopsy. Baseline pulmonary function data (mean +/- SEM percent predicted) were as follows: total lung capacity, 57.3 +/- 2.5; vital capacity, 55.0 +/- 3.3; diffusing capacity of lung for carbon monoxide, 39.7 +/- 2.8; and forced expiratory volume in 1 second, 58.1 +/- 3.6. Only 1 patient showed symptomatic and functional improvement, and 7 discontinued treatment because of a perceived lack of benefit. Eleven patients (52%) died after a mean of 6.4 months of treatment, and follow-up pulmonary function data suggested continued worsening in all but 1 patient. CONCLUSION: These observations do not support the use of interferon gamma-lb therapy for patients with advanced IPF/UIP.

Aged↗