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Jay H Ryu

Publications and source records attributed to Jay H Ryu.

At least 19 recordsLinked to original sources
PubMed2006

Organizing pneumonia in patients with hematologic malignancies: a steroid-responsive lesion.

BACKGROUND: Organizing pneumonia (OP) is a distinct histopathologic lesion that occurs in a variety of clinical contexts. There have been occasional reports of OP occurring in patients with hematologic malignancies. STUDY OBJECTIVES: To examine the association of OP with hematologic malignancies and to assess the clinical course of affected patients. DESIGN: A retrospective review of our institutional experience of unexplained OP developing in patients with hematologic malignancies. SETTING: Tertiary care, referral medical center. PATIENTS: We identified 6 patients with a verified histopathologic diagnosis of OP and antecedent or concomitant diagnosis of a hematologic malignancy from the Mayo Clinic database (1995-2003). Clinical, radiologic, and outcome data were abstracted from records. RESULTS: Underlying hematologic disorders included lymphoma (2), acute leukemia (2), cutaneous T-cell lymphoma (1), and myelodysplastic syndrome (1). OP was diagnosed by surgical lung biopsy in 4 and bronchoscopic biopsy in 2. Four of the 6 patients had previously been exposed to chemotherapeutic agents, two had not. Three of the 6 patients had bone marrow transplantation prior to development of OP. Five patients were treated with prednisone and all experienced symptomatic improvement with documented radiologic resolution in 4. One patient experienced symptomatic and radiologic resolution with observation alone. Three patients ultimately died from complications of their underlying hematologic disorder and 1 patient died of unknown causes. Two patients were alive without respiratory complaints more than 1 year after lung biopsy. CONCLUSIONS: OP occurs in patients with underlying hematologic malignancies who may or may not have been treated with chemotherapy and responds favorably to corticosteroid therapy.

Acute Disease↗
PubMed2006

Morphologic and quantitative assessment of CD20+ B cell infiltrates in rheumatoid arthritis-associated nonspecific interstitial pneumonia and usual interstitial pneumonia.

OBJECTIVE: B lymphocytes are emerging as important elements in the events leading to joint destruction in rheumatoid arthritis (RA). However, B lymphocytes have not been studied in rheumatoid arthritis (RA)-associated lung disease. We performed a morphologic and quantitative analysis of B lymphocytes and plasma cells in RA-associated interstitial pneumonia (IP) in comparison with idiopathic IP and normal lungs. METHODS: Open-lung biopsy specimens from patients with RA-associated IP (n = 18), patients with idiopathic IP (n = 21), and control subjects (n = 11) were stained with antibodies to CD20 and CD138. Morphologic patterns of stained specimens were characterized and staining was quantified using computer-assisted image analysis. RESULTS: In RA-associated IP, marked follicular B cell hyperplasia was detected, which was limited almost entirely to peribronchiolar lymphoid aggregates. Plasma cells were also present in large numbers, but showed a more diffuse tissue infiltration. Quantification of B cells demonstrated higher cellularity in RA-associated IP (median 2.0%, interquartile range [IQR] 1.0-5.7) as compared with idiopathic IP (0.9%, IQR 0.5-2.1). Control specimens showed a significantly smaller number of B cells compared with both diseases (0.4%, IQR 0.1-1.3). In RA patients who were smokers and in those who were male, the proportion of CD20+ tissue areas further increased to 4.3% (IQR 1.0-5.8) and 3.9% (IQR 0.7-6.9), respectively. CONCLUSION: We demonstrated a significant follicular B cell hyperplasia in RA-associated IP. The differences between RA-associated IP and idiopathic IP imply a differential emphasis of B cell-mediated mechanisms in the 2 diseases despite radiologic and histologic similarities and provide a rationale for studying functional aspects of B cell involvement in the pathogenesis of RA-associated IP.

Adult↗
PubMed2006

Microscopic polyangiitis associated with thymoma, exacerbating after thymectomy.

A variety of autoimmune diseases has been associated with thymoma, and thymectomy does not always induce remission of these disorders. This case report describes a 50-year-old man who presented with migratory polyarthritis and an anterior mediastinal mass that proved to be a thymoma. Five months after thymectomy, the patient presented with worsening polyarthritis, hematuria, and azotemia. Based on elevated titers of antineutrophil cytoplasmic antibodies directed against myeloperoxidase and renal biopsy showing crescentic necrotizing glomerulonephritis, microscopic polyangiitis was diagnosed. After remission-induction therapy with prednisone and cyclophosphamide, articular symptoms and renal manifestations resolved. Microscopic polyangiitis was not associated previously with thymoma, and this case broadens the spectrum of autoimmune disorders seen with this tumor. Progressive disease seen after thymectomy in this patient has potential implications regarding the pathophysiological characteristics of microscopic polyangiitis and management of patients with this clinical association.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed2006

Treatment of idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF) is a relentlessly progressive lung disease in most cases, and effective treatment is still lacking. This review examines the current status of treatment options and complexities in the management of patients with IPF. Although optimal therapy for IPF has not been identified, ongoing research efforts warrant reason for optimism. Current management of IPF includes not only judicious use of available pharmacological agents tailored to individual circumstances but also patient education through realistic assessment of prognosis, discussion of pros and cons of pharmacotherapy, early consideration of lung transplantation when applicable, treatment of complications, supportive care, and encouragement to participate in clinical trials.

Adrenal Cortex Hormones↗
PubMed2006

Bronchogenic cyst causing atrial fibrillation by impinging the right inferior pulmonary vein.

Atrial fibrillation is the most common sustained cardiac arrhythmia and is usually associated with underlying structural heart disease, but may also occur in isolation--the entity of "lone" atrial fibrillation. Recently, attention has been directed to the pulmonary veins as a source of the arrhythmia through identification of rapidly firing ectopic foci within the covering myocardial sleeves. We describe a 38-year-old man who presented with treatment-resistant atrial fibrillation and a posterior mediastinal mass. Electrophysiological studies demonstrated abnormal foci of electrical activity at the entrance of the right inferior pulmonary vein into the left atrium. Surgical exploration revealed a bronchogenic cyst that distorted and stretched the right inferior pulmonary vein as it traversed the posterior mediastinum towards the left atrium. Restoration of sinus rhythm without recurrence of atrial fibrillation characterized the clinical course after surgical resection of the mass. This case demonstrates that a retro-cardiac bronchogenic cyst can cause atrial fibrillation by impinging on a pulmonary vein.

Adult↗
PubMed2006

Classification and approach to bronchiolar diseases.

PURPOSE OF REVIEW: Bronchiolar abnormalities are relatively common and occur in a variety of clinical contexts. There have been an increasing number of terms, some of which are redundant, used in referring to various forms of bronchiolar disorders. The purpose of this review is to provide an updated classification scheme to facilitate the clinical approach to patients with suspected bronchiolar disease. RECENT FINDINGS: The spectrum of bronchiolar disorders is broader and more heterogeneous than previously recognized. In deciphering the potential significance of bronchiolar abnormalities, it is useful to distinguish those disorders in which the bronchiolar involvement is the dominant pathologic process, that is, primary bronchiolar disorders, from parenchymal or large airway diseases in which the bronchioles may become secondarily involved. Primary bronchiolar disorders include respiratory bronchiolitis, acute bronchiolitis, constrictive bronchiolitis, follicular bronchiolitis, diffuse panbronchiolitis, mineral dust airway disease, and a few other variants. Interstitial lung diseases with a prominent bronchiolar involvement include smoking-related interstitial lung diseases, cryptogenic organizing pneumonia, and hypersensitivity pneumonitis. Bronchiolitis can also be seen in large airway diseases such as chronic bronchitis, bronchiectasis, and asthma. SUMMARY: In the clinical approach to a patient with bronchiolar disease, primary bronchiolar disorders should be distinguished from predominantly parenchymal or large airway processes with bronchiolar involvement. The number of patterns of bronchiolar response to injury is limited and these patterns are generally non-specific in regard to cause. Appropriate diagnosis and management of patients with bronchiolar disorders depend on judicious correlation of clinical, physiologic, and morphologic manifestations.

Airway Obstruction↗
PubMed2006

Management of pneumothorax in lymphangioleiomyomatosis: effects on recurrence and lung transplantation complications.

STUDY OBJECTIVES: Pneumothorax is a common complication of lymphangioleiomyomatosis (LAM), and the optimal approach to its treatment and prevention is unknown. Chemical or surgical pleurodesis are often required to prevent recurrence. However, their efficacy in LAM is unclear, and whether they contribute to perioperative complications during lung transplantation is uncertain. SETTING: The LAM Foundation database of registered patients. DESIGN: A questionnaire was sent to all registered patients who had at least one pneumothorax to determine rates and patterns of recurrence and efficacy of interventions. A second questionnaire was sent to registered LAM patients who received a lung transplant. PATIENTS OR PARTICIPANTS: Of 395 registered patients, 260 patients (66%) reported at least one pneumothorax during their lifetime, 193 of whom (74%) completed the questionnaire. Of the 85 lung transplant patients who were sent a separate questionnaire, 80 patients (94%) responded. INTERVENTIONS: None. MEASUREMENTS AND RESULTS: Of the 193 respondents to the pneumothorax questionnaire, data on 676 episodes of pneumothorax were collected. Eighty-two percent (158 of 193 patients) had their first pneumothorax prior to a diagnosis of LAM. One hundred forty patients (73%) had at least one additional pneumothorax, either an ipsilateral recurrence (99 of 140 patients, 71%) or a contralateral pneumothorax (104 of 140 patients, 74%). Recurrence rates were 66% after conservative therapy, 27% after chemical pleurodesis, and 32% after surgery. In patients who had undergone lung transplantation, prior chemical or surgical pleurodesis was performed in 45 of 80 patients (56%). Fourteen of 80 patients (18%) reported pleural-related postoperative bleeding, 13 of whom (93%) had prior pleurodesis. CONCLUSIONS: Chemical pleurodesis or surgery are equally effective and better than conservative therapy in preventing recurrence of pneumothorax in LAM. Due to the high recurrence rate, either procedure should be considered for the initial pneumothorax in these patients. However, both contribute to increased perioperative bleeding following lung transplantation, with no effect on length of hospital stay.

Adolescent↗
PubMed2006

Diffuse alveolar damage: uncommon manifestation of pulmonary involvement in patients with connective tissue diseases.

BACKGROUND: Diffuse alveolar damage (DAD) is a relatively common finding on surgical lung biopsy and can result from a variety of causes. METHODS: We studied nine consecutive patients with connective tissue disease (CTD) and DAD diagnosed on surgical lung biopsy to examine this association and clinical implications. RESULTS: The median age was 63 years (range, 35 to 76 years), and seven of the patients were women (78%). Underlying CTDs included rheumatoid arthritis in five patients, polymyositis in two patients, and one patient each with systemic sclerosis and mixed CTD. In seven patients (78%), CTD had been diagnosed before the onset of DAD; six of these patients had a preexisting interstitial lung disease (ILD) related to their CTD. DAD was the presenting manifestation leading to a new CTD diagnosis in two patients (22%). CT of the chest revealed ground-glass opacities and/or consolidation bilaterally with or without honeycombing. In all patients, surgical lung biopsy revealed DAD for which no cause could be identified other than the underlying CTD. Seven patients (78%) were receiving mechanical ventilatory support at the time of the surgical lung biopsy. Four patients (44%) survived to hospital discharge and included one patient with preexisting ILD and all three patients without chronic ILD. CONCLUSION: We conclude that DAD can complicate the clinical course of patients with CTD-related chronic ILD, or can occasionally occur as a presenting manifestation of CTDs. When DAD occurs in patients with CTDs, the outcome appears to be worse for those with preexisting chronic ILD compared to those without ILD.

Adult↗
PubMed2006

Interstitial lung disease in primary Sjögren syndrome.

BACKGROUND: Primary Sjögren syndrome (pSS) has been associated with various histologic patterns of interstitial lung disease (ILD). METHODS: We retrospectively identified 18 patients with pSS and suspected ILD who underwent lung biopsies (14 surgical biopsies and 9 bronchoscopic biopsies) at our institution during a 13-year period from 1992 through 2004. Histopathologic findings were analyzed and correlated with radiologic features and outcome. RESULTS: Median age was 62 years (range, 34 to 78 years), and 15 patients (83%) were women. Most patients presented with dyspnea and cough. Chest radiographs demonstrated bilateral infiltrates, and high-resolution CT revealed abnormalities of various types including ground-glass, consolidation, reticular, and nodular opacities. The major histopathologic patterns included nonspecific interstitial pneumonia (NSIP) [five patients], organizing pneumonia (OP) [four patients], usual interstitial pneumonia (UIP) [three patients], lymphocytic interstitial pneumonia (three patients), primary pulmonary lymphoma (two patients), and diffuse interstitial amyloidosis (one patient). In four patients (three with OP and one with amyloidosis), the diagnosis was established on transbronchial biopsy results. Treatment commonly included prednisone with or without another immunosuppressive agent. During the follow-up period (median, 38 months), most patients improved or remained stable except three patients with UIP, one patient with NSIP, and one patient with amyloidosis. Seven patients (39%) died, including three deaths from acute exacerbation of interstitial pneumonia. CONCLUSIONS: A variety of histologic patterns can be seen in patients with pSS-associated ILD. Those with UIP tended to have progression of lung disease. Death from acute exacerbation of interstitial pneumonia may occur in patients with pSS-associated ILD.

Adrenal Cortex Hormones↗
PubMed2006

Diffuse bronchiolar disease due to chronic occult aspiration.

OBJECTIVE: To describe clinical, radiological, and histopathologic features of diffuse bronchiolar disease due to chronic occult aspiration. PATIENTS AND METHODS: We identified 4 patients encountered from July 2001 to January 2004 who had persistent respiratory symptoms and lung Infiltrates and who were eventually diagnosed by surgical lung biopsy as having diffuse bronchiolar disease due to chronic occult aspiration. Medical records, radiological studies, and histopathologic specimens were reviewed to assess their clinicoradiologic presentation and diagnostic features. RESULTS: The mean age of these 4 patients was 50 years (age range, 41-59 years), and 2 were women. All presented with persistent dyspnea, cough, and lung infiltrates. Three had a history of gastroesophageal reflux, but only 1 had active symptoms. Chest radiography showed interstitial infiltrates, whereas the predominant finding on computed tomography was numerous centrilobular nodules in all patients. Bronchoscopic lung biopsies had been performed in all patients, and the results were nondiagnostic. Surgical lung biopsy specimens revealed diagnostic features that consisted of bronchiolocentric organizing pneumonia with giant cells that contained material consistent with food in all 4 patients. CONCLUSION: Diffuse bronchiolar disease likely represents an underrecognized form of aspiration-related lung disease and may occur in relatively young Individuals without symptoms suggestive of recurrent aspiration. Radiological features associated with this disorder are distinctively different from those seen in aspiration pneumonia.

Adult↗
PubMed2005

The NHLBI lymphangioleiomyomatosis registry: characteristics of 230 patients at enrollment.

RATIONALE: Pulmonary lymphangioleiomyomatosis is a progressive cystic lung disease that is associated with infiltration of atypical smooth muscle-like cells. Previous descriptions of clinical characteristics of subjects with lymphangioleiomyomatosis have been based on a limited number of patients. OBJECTIVES: To describe the clinical characteristics of subjects with pulmonary lymphangioleiomyomatosis, both sporadic and tuberous sclerosis-related forms. METHODS: Over a 3-yr period, from 1998 to 2001, 243 subjects with pulmonary lymphangioleiomyomatosis were enrolled into a national registry; 13 subjects who had already undergone lung transplantation were excluded for the purposes of this report. MEASUREMENTS AND MAIN RESULTS: All 230 subjects were women, aged 18 to 76 yr (mean +/- SE, 44.5 +/- 0.65 yr). The average age at onset of symptoms was 38.9 +/- 0.73 yr and at diagnosis was 41.0 +/- 0.65 yr. Tuberous sclerosis complex was present in 14.8% of subjects. Pulmonary manifestations, most commonly spontaneous pneumothorax, were the primary events leading to the diagnosis in 86.5% of cases. Nearly 55% of the subjects were being treated with a progesterone derivative. An obstructive pattern on pulmonary function testing was observed in 57.3% of the subjects, whereas 33.9% had normal spirometric results. Women with tuberous sclerosis-related lymphangioleiomyomatosis were younger and had less impaired lung function compared with those with the sporadic form. CONCLUSIONS: The age range of women afflicted with pulmonary lymphangioleiomyomatosis is broader than previously appreciated and the degree of pulmonary function can be quite variable, with one-third of subjects having normal spirometry at enrollment into this registry.

Adolescent↗
PubMed2005

Hot tub lung: presenting features and clinical course of 21 patients.

BACKGROUND: Hot tub lung is an emerging lung disorder associated with exposure to Mycobacterium avium complex organisms contaminating hot tub water. OBJECTIVES: To define the clinical characteristics and outcome of patients with hot tub lung. METHODS: Retrospective review of 21 patients diagnosed with hot tub lung at a tertiary medical center over a 7-year period. RESULTS: The mean (+/-sd) age at presentation was 46 (+/- 15) years; 9 patients were men (43%). All patients described ongoing exposure to hot tubs. The most common referral diagnoses were sarcoidosis, bronchitis, and asthma. Dyspnea and cough were present in all patients, hypoxemia was noted in 10 patients (48%). High-resolution computed tomography of the chest had been performed in 20 patients and demonstrated diffuse centrilobular nodules and/or ground-glass opacities in all patients. M. avium complex was isolated from the hot tub water, respiratory secretions and/or lung tissue in all patients. Bronchoscopic or surgical lung biopsy was obtained in 18 patients and demonstrated bronchiolocentric granulomatous inflammation. With avoidance of exposure, clinical and radiologic improvement was observed in all patients. Additionally, 13 patients (62%) received corticosteroid therapy, 1 (5%) antimycobacterial therapy, 2 (10%) received both, and 5 patients (24%) received no pharmacologic therapy. CONCLUSIONS: Hot tub lung likely represents hypersensitivity pneumonitis due to inhalational exposure to M. avium complex. Antimycobacterial therapy does not appear to be required in the management of this disease. Although corticosteroids may be helpful in the treatment of severely affected patients, others can be managed by avoidance of additional exposure alone.

Adrenal Cortex Hormones↗
PubMed2005

Sonographically guided thoracentesis and rate of pneumothorax.

PURPOSE: To assess whether thoracenteses performed with sonographic guidance are associated with a lower rater of pneumothorax and tube thoracostomy than those performed without sonographic guidance. METHODS: We reviewed the medical records of 523 subjects undergoing their initial diagnostic thoracentesis at our institution from July 1, 2001, to June 30, 2002. We excluded 73 subjects in whom no chest imaging had been performed within 5 days of thoracentesis or who had pre-existing chronic hydropneumothorax. RESULTS: Of the 450 thoracenteses performed, 305 (67.8%) were performed with sonographic guidance and 145 (32.2%) were performed without. On postthoracentesis imaging in all subjects, 30 pneumothoraces (6.7%) were found (23 inpatients, 7 out-patients). Eight patients required a tube thoracostomy for their pneumothorax. Pneumothorax occurred in 15 of 305 procedures (4.9%) performed with sonographic guidance and 15 of 145 procedures (10.3%) performed without (p < 0.05). Tube thoracostomy was performed in 0.7% of patients whose thoracentesis was performed with sonographic guidance and in 4.1% in those that were not (p < 0.05). We found no correlation between pneumothorax after thoracentesis and age, inpatient status, loculation of effusion, or volume of pleural fluid removed. CONCLUSIONS: The routine use of sonography during diagnostic thoracentesis is associated with a reduced rate of pneumothorax and tube thoracostomy.

Adolescent↗
PubMed2005

Usual interstitial pneumonia complicating dyskeratosis congenita.

Dyskeratosis congenita (DC) is a rare disorder characterized by skin hyperpigmentation, nail dystrophy, and leukoplakia of mucous membranes. Pulmonary complications occur in approximately 20% of patients, although the specific histopathologic features, the temporal relationship between the diagnosis of DC and the development of pulmonary fibrosis, and the response to specific treatment are largely undefined. We describe 2 patients with DC who developed usual interstitial pneumonia. Pulmonary fibrosis developed 18 and 38 years after the original manifestations of DC. Both patients died of respiratory failure, 4 and 6 months after lung biopsy. Pulmonary fibrosis in patients with DC may be linked to underlying abnormalities of fibroblast function.

Adult↗
PubMed2005

Idiopathic pulmonary fibrosis in patients younger than 50 years.

OBJECTIVE: To assess clinical, radiological, histopathologic, and prognostic differences in younger patients with idiopathic pulmonary fibrosis (IPF). PATIENTS AND METHODS: This study consisted of patients younger than 50 years with IPF who were seen at the Mayo Clinic in Rochester, Minn, from January 1, 1994, to December 31, 2000. Clinical, radiological, and histopathologic data were abstracted from clinical records. Total lung capacity using plethysmography, vital capacity, diffusing capacity for carbon monoxide using the single-breath method, and alveolar volume using single-breath neon wash-in were measured. RESULTS: Our study population was composed of 16 men and 6 women with a median age of 45 years (range, 28-49 years). Median survival was 2.1 years, with 1- and 2-year survival rates of 68% and 53%, respectively. Of the 22 patients, 2 were current smokers, 14 were former smokers, and 6 had never smoked. Three patients had familial pulmonary fibrosis, 21 had bibasilar inspiratory crackles, and 10 had digital clubbing. Median total lung capacity was 56.2%, vital capacity was 51.0%, diffusing capacity was 45.5%, and alveolar volume was 65.0%. CONCLUSION: Although previous studies have suggested that younger age is a favorable prognostic factor in patients with IPF, we found that younger patients have the same poor prognosis as do older patients with this disorder. We observed no distinguishing differences in the clinical, radiological, and histopathologic features compared with those in older patients in whom the disorder is more common. Lung transplantation should be considered early in the treatment of younger patients with IPF.

Adult↗
PubMed2005

Pulmonary cryptococcosis: CT findings in immunocompetent patients.

PURPOSE: To evaluate retrospectively the computed tomographic (CT) findings in immunocompetent patients with pulmonary cryptococcosis. MATERIALS AND METHODS: Institutional review board approval was obtained with a waiver of informed consent, and the study complied with requirements of the Health Insurance Portability and Accountability Act. Chest CT scans of 10 immunocompetent patients with clinically proved pulmonary cryptococcosis were retrospectively reviewed by four reviewers in consensus. Criterion for diagnosis of pulmonary cryptococcosis was (a) the histopathologic presence of the organism at lung biopsy or (b) a positive culture of a respiratory specimen or positive serum cryptococcal antigen test with clinical or radiographic evidence of active pulmonary infection. Patients included six women and four men ranging in age from 46 to 73 years (mean, 59 years). Scans were evaluated for nodules, masses, areas of ground-glass attenuation or of hazy increased attenuation, areas of consolidation, areas of cavitation, pleural effusions, linear opacities, septal thickening, lymphadenopathy, extent of parenchymal involvement, and distribution. RESULTS: The most common CT finding was pulmonary nodules (n = 9). Multiple nodules (n = 7) were more common than solitary nodules (n = 2). Nodules most commonly occupied less than 10% of the pulmonary parenchyma (n = 7), measured less than 10 mm in diameter (n = 7), and had middle and upper lung predominance (n = 6). The majority of the nodules were well defined with smooth margins (n = 7). Multiple nodules were usually bilaterally distributed (n = 5). Masses (n = 2), lymphadenopathy (n = 2), areas of consolidation (n = 2), areas of hazy increased attenuation (n = 1), pleural effusion (n = 1), and areas of cavitation (n = 1) were uncommon. CONCLUSION: CT most commonly demonstrated pulmonary nodules in immunocompetent patients with pulmonary cryptococcosis. The nodules were most often multiple, small, well defined, and smoothly marginated with middle and upper lung predominance.

Aged↗