Body mass index and mortality in patients with idiopathic pulmonary fibrosis.

BACKGROUND: To examine the relationship between body mass index (BMI) and mortality in patients with idiopathic pulmonary fibrosis (IPF). METHODS: We studied a cohort of patients with IPF who were seen at the Mayo Clinic Rochester from 1994 through 1996. These patients met the current consensus definition of IPF. We excluded patients who had received prior treatment for IPF, had no follow-up data, or had no pulmonary function results available at the index visit. RESULTS: Of the 197 patients fulfilling the inclusion criteria, the mean (+/- SD) age was 71.4 +/- 8.9 years, 137 patients (70%) were men, and the mean BMI was 28.2 +/- 4.6. These patients were categorized by BMI into the following three groups: < 25; 25 to 30; and >/= 30. There were 46 patients (23%) with a BMI of < 25 who had a median survival time of 3.6 years (1-year survival rate, 76% [95% confidence interval (CI), 65 to 90%]; 3-year survival rate, 54% [95% CI, 41 to 70%]). The second group consisted of 85 patients (43%) with a BMI between 25 and 30 who had a median survival time of 3.8 years (1-year survival rate, 84% [95% CI, 76 to 92%]; 3-year survival rate, 58% [95% CI, 48 to 70%]). The final group consisted of 66 patients (34%) with a BMI of >/= 30 and who had a median survival time of 5.8 years (1-year survival rate, 91% [95% CI, 84 to 98%]; 3-year survival rate, 69% [95% CI, 58 to 81%]). Using a proportional hazards regression model, survival was significantly associated with BMI (hazard ratio, 0.93 for each 1-U increase in BMI; 95% CI, 0.89 to 0.97; p = 0.002) with increased BMI being associated with better survival. CONCLUSION: Higher BMI was associated with better survival in patients with IPF.

Chronic nitrofurantoin-induced lung disease.

OBJECTIVE: To reassess the clinical and radiological features of chronic nitrofurantoin-induced lung disease and eventual clinical outcome. PATIENTS AND METHODS: We retrospectively reviewed the medical records of 18 patients with chronic nitrofurantoin-induced lung disease who were seen at the Mayo Clinic in Rochester, Minn, from January 1, 1997, to December 31, 2002. RESULTS: The median age of the 18 patients was 72 years (range, 47-90 years) at the time of diagnosis; 17 (94%) were women. Onset of symptoms occurred after a median interval of 23 months (range, 10-144 months) following the initiation of nitrofurantoin therapy for the prevention of recurrent urinary tract infections. All patients presented with persistent dyspnea and cough associated with lung infiltrates detected on chest radiography. Ten computed tomograms were available for review and revealed bilateral areas of ground-glass opacities in all cases and showed subpleural Irregular linear opacities and patchy consolidation in some cases. Nitrofurantoin therapy was discontinued in all patients, and most improved subsequently; 9 patients received corticosteroid therapy. CONCLUSIONS: Chronic nitrofurantoin-induced lung disease is seen predominantly in older women who present with respiratory symptoms after a year or more of nitrofurantoin therapy. Associated radiological features are relatively nonspecific but usually include bilateral areas of ground-glass opacities on computed tomography of the chest. Cessation of nitrofurantoin therapy leads to improvement and suffices in the management of some patients, although corticosteroid therapy may be helpful in those more severely affected.

Pulmonary hypertension in patients with idiopathic pulmonary fibrosis.

STUDY OBJECTIVES: To determine the impact on survival and clinical correlates of pulmonary hypertension (PH) occurring in patients with idiopathic pulmonary fibrosis (IPF). DESIGN: Retrospective study. SETTING: Tertiary care, referral medical center. PATIENTS: Among 487 consecutive patients with IPF, we identified 136 patients who underwent transthoracic echocardiography within 3 months of their initial evaluation at our institution. Patients with left ventricular dysfunction, valvular heart disease, incomplete follow-up, and those in whom pulmonary artery pressures could not be assessed were excluded; the remaining 88 patients were included in this study. Correlations were performed between echocardiographic measures of PH and clinical variables including survival. MEASUREMENTS AND RESULTS: The mean (+/- SD) estimated systolic pulmonary artery pressure (SPAP) for the 88 patients was 48 +/- 16 mm Hg (range, 28 to 116 mm Hg). Among pulmonary function parameters, SPAP correlated best with diffusing capacity of the lung for carbon monoxide (D(LCO)), to which it was inversely related. For survival analysis, patients were stratified into three groups: < or = 35 mm Hg (14 patients), 36 to 50 mm Hg (47 patients), and > 50 mm Hg (27 patients). Using the Kaplan-Meier method, the median survival rates for these three groups were 4.8 years, 4.1 years, and 0.7 years, respectively. Those patients with SPAP > 50 mm Hg had significantly worse survival compared to other subgroups (p = 0.009). CONCLUSION: In patients with IPF, PH correlates inversely with D(LCO) and has a significant adverse impact on survival, particularly when SPAP is > 50 mm Hg.

Pulmonary complications in adult blood and marrow transplant recipients: autopsy findings.

STUDY OBJECTIVE: To describe the pulmonary findings at autopsy of blood and bone marrow transplant (BMT) recipients. DESIGN: Retrospective. SETTING: An academic medical center. PATIENTS: Seventy-one deceased adult BMT recipients. INTERVENTIONS: None. MEASUREMENTS: Antemortem and postmortem pulmonary findings. RESULTS: The transplants were allogeneic in 39 patients (55%), with a peripheral stem cell source in 43 patients (61%). Death occurred at a median of 1.30 months after transplant. Ninety-six pulmonary complications were noted in 63 patients (89%): 27 infectious (bacterial bronchopneumonia, n = 13; pulmonary aspergillosis, n = 11; cytomegalovirus pneumonia, n = 2; and Candida bronchopneumonia, n = 1) and 69 noninfectious (diffuse alveolar damage, n = 35; diffuse alveolar hemorrhage [DAH], n = 10; amyloidosis, n = 9; pulmonary embolism, n = 5; lymphoma/leukemia, n = 4; bronchiolitis obliterans, n = 2; bronchiolitis obliterans organizing pneumonia, n = 1; pulmonary alveolar proteinosis, n = 1; aspiration pneumonia, n = 1; and acute and organizing pneumonia, n = 1). Twenty-seven of the 96 complications (28%) were diagnosed antemortem. Infectious complications were more likely to be diagnosed antemortem compared to noninfectious complications (48% vs 20%, p = 0.006). Six of the 13 patients with bronchopneumonia (46%), 5 of the 11 patients with pulmonary aspergillosis (45%), and 7 of the 8 patients with DAH (88%) at autopsy were not receiving treatment for these conditions at the time of death. Ten patients being treated for suspected pulmonary aspergillosis, 7 patients treated for suspected pulmonary cytomegalovirus infection, 22 patients treated for suspected bacterial pneumonia, 2 patients treated for suspected Pneumocystis carinii pneumonia, and 12 patients treated for DAH at the time of death had no evidence of these conditions at autopsy. The most common immediate cause of death was respiratory failure (n = 37, 52%). CONCLUSIONS: Pulmonary complications, the majority not diagnosed antemortem, are the most common cause of death in BMT recipients. As the result of underdiagnosis, BMT recipients may not receive appropriate therapy for potentially treatable pulmonary complications.

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.

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.

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.

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.

Tracheal myxoma: a rare benign tracheal tumor.

Benign tracheal tumors are rare. We describe a 39-year-old man who underwent resection of a tracheal myxoma, a previously unrecognized benign tracheal neoplasm. He presented with a 9-month history of wheezing, cough, and dyspnea on exertion. Treatment with bronchodilators and corticosteroids administered by inhalation and systemically did not diminish his symptoms. Pulmonary function tests showed a pattern of airflow limitation consistent with variable extrathoracic obstruction. Chest radiography and computed tomography revealed a tracheal mass. Tracheal resection of the tumor with reconstruction was curative. The patient is free of disease 7 years after surgery.

Rare tracheal chondroid hamartoma masquerading as asthma in a 14-year-old girl.

BACKGROUND: Tracheal hamartomas are rare in all age groups and have not been previously described in adolescence. OBJECTIVE: To report the first case of a tracheal chondroid hamartoma presenting as exercise intolerance and wheezing and previously misdiagnosed and treated as asthma. METHODS: Symptoms, pulmonary function tests, chest x-ray examination, chest computed tomography, and histologic examination of the specimen were performed. RESULTS: The pulmonary function tests obtained throughout several years revealed progressive decreases (approximately 30% of predicted) in peak expiratory flow and forced expiratory volume in 1 second (approximately 50% of predicted). The inspiratory and expiratory flow-volume curve suggested a fixed central airway obstruction. Both the chest x-ray examination and computed tomography revealed an intraluminal tracheal tumor that was surgically excised. Histologic examination revealed a chondroid hamartoma. CONCLUSIONS: Rare benign primary tracheal tumors, including chondroid hamartoma, can present in adolescence with asthma-like symptoms for years and should be considered in the differential diagnosis, especially in the setting of appropriately abnormal spirometry or when asthma is very difficult to control.

Agnogenic myeloid metaplasia with pleural extramedullary leukemic transformation.

Agnogenic myeloid metaplasia (AMM) is one of the myeloproliferative disorders, and is usually accompanied by extramedullary hematopoiesis (EMH) in various organs, mainly in the liver, spleen and lymph nodes. Extramedullary hematopoiesis and/or leukemic transformation of EMH in the pleura is a rare occurrence and is usually asymptomatic. Pleural involvement is usually diagnosed on postmortem examination. Herein we describe a 71-year-old man with newly diagnosed agnogenic myeloid metaplasia who was evaluated for progressively worsening dyspnea, pulmonary hypertension and bilateral pleural effusions. EMH involving the lungs and pleura was suspected. A sulfur colloid technetium 99m bone marrow scan performed to detect extramedullary hematopoiesis was negative. The diagnostic thoracentesis yielded bloody fluid that contained a large population of myeloblasts, indicating pleural leukemic transformation. The patient received 100 cGy to the whole lung for treatment of pulmonary hypertension due to EMH. This was followed by 1500 cGy total dose of radiation to the left lung for pleural extramedullary leukemic transformation. Pleural effusions resolved and repeat echocardiography showed reduction of the pulmonary artery pressure. Three months later he had leukemic transformation involving the skin and lymph nodes. Four months after radiation therapy, he had full-blown acute myeloid leukemia. He received 2 cycles of Gemtuzumab ozogamicin (Mylotarg), allopurinol and hydroxyurea. Three months after initiation of chemotherapy, he deteriorated and received salvage chemotherapy of prednisone, VP-16 and imatinib mesylate (Gleevec). He was hospitalized for neutropenic fever and was diagnosed to have pulmonary aspergillosis. He died of multisystem failure 8 1/2 months after being diagnosed with AMM.

Pneumothorax in pulmonary Langerhans cell histiocytosis.

BACKGROUND: Pulmonary Langerhans cell histiocytosis (PLCH) is a smoking-related interstitial lung disease characterized by development of cystic changes that predispose to occurrence of pneumothorax. STUDY OBJECTIVES: To determine the frequency, recurrence rate, and optimal management of pneumothorax associated with PLCH. DESIGN: Retrospective study. SETTING: Tertiary care, referral medical center. PATIENTS: One hundred two adults >or= 18 years old with histologically confirmed PLCH seen at Mayo Clinic Rochester over a 23-year period from 1976 to 1998. INTERVENTIONS: None. MEASUREMENTS AND RESULTS: Sixteen of 102 patients (16%) with PLCH had pneumothorax; mean age at the time of diagnosis was 29.4 years (range, 18 to 52 years), and all had smoked cigarettes. There were 37 episodes of pneumothoraces (1 to 5 episodes per patient); 10 patients (63%) had more than one episode. Median age at diagnosis of PLCH was significantly younger in patients with pneumothorax when compared to those without pneumothorax (27 years vs 41.5 years, p < 0.001), but pulmonary function parameters and survival after diagnosis were not significantly different. Rate of recurrent pneumothorax was 58% to the ipsilateral side when the episode was managed by observation or chest tube without pleurodesis, and 0% after surgical management with pleurodesis. CONCLUSIONS: These data support the early use of surgical therapy with pleurodesis in managing patients with PLCH and spontaneous pneumothorax.

Cryptococcal laryngitis: case report and review of the literature.

Infections caused by Cryptococcus neoformans may range from an asymptomatic illness to lethal systemic disease, especially in immunocompromised hosts. Although cryptococcal infection most commonly involves the lungs or the central nervous system, it can disseminate to virtually any organ. Laryngeal cryptococcal infections are extremely rare--only 5 cases have been reported in the literature. Herein, we describe cryptococcal laryngitis occurring in a 55-year-old man with asthma and allergic fungal sinusitis. He presented with hoarseness and cough. He was treated with itraconazole followed by fluconazole therapy with complete recovery from his laryngeal infection. The patient was disease-free when last seen 10 months after the diagnosis. We present a summary of all reported cases of cryptococcal laryngitis.

Glomus tumor of the trachea: value of multidetector computed tomographic virtual bronchoscopy.

Glomus tumor of the trachea is extremely rare. We report a case of tracheal glomus tumor in a 39-year-old man who presented with hemoptysis. The diagnosis was made after bronchoscopic biopsy of a tumor involving the posterior wall of the upper trachea. Thin-section multidetector computed tomography of the chest was performed before surgical resection, with multiplanar re-formations and 3-dimensional virtual bronchoscopic reconstruction. Tracheal sleeve resection with reconstruction was successful, and pathological studies confirmed complete resection and the diagnosis of glomus tumor. The patient was disease-free 3 months postoperatively. To our knowledge, this is the first reported case in which additional computed postprocessing was used to help evaluate the extent of such a tumor.

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.

The role of autopsy in the intensive care unit.

OBJECTIVE: To identify the frequency and spectrum of clinically relevant diagnoses found at autopsy but not determined before death in adult patients admitted to an intensive care unit (ICU). PATIENTS AND METHODS: We retrospectively reviewed medical records and autopsy reports of patients admitted to ICUs from January 1, 1998, to December 31, 2000. Disagreements between autopsy and antemortem diagnoses were classified as type I or type II errors. A new major diagnosis with potential for directly impacting therapy was considered a type I error. Type II errors included important findings that would not have likely changed therapy. RESULTS: Of 1597 deaths in all ICUs during the study period, autopsies were performed in 527 patients (33%). Autopsy reports were available in 455 patients, of whom 19 (4%) had type I errors and 78 (17%) had type II errors. The most common type I error was cardiac tamponade. There were no significant differences in age, sex, or length of stay in the ICU or hospital among patients with and without diagnostic errors or among patients with type I and II errors. Seventy-eight patients had 81 type II errors. Organ transplant recipients had more type I or II errors than did nontransplant patients (35% vs 20%; P = .04). CONCLUSIONS: Diagnoses with impact on therapy and outcome are missed in approximately 4% of deaths of adult patients admitted to the ICU. Transplant recipients are especially likely to have occult conditions for which additional therapy might be indicated.