Interstitial lung disease in classic and skin-predominant dermatomyositis: a retrospective study with screening recommendations.

OBJECTIVES: (1) To determine the prevalence of interstitial lung disease (ILD) and isolated low diffusing capacity for carbon monoxide (DLCO) in a large cohort of outpatients with dermatomyositis. (2) To compare the pulmonary abnormalities of patients with classic dermatomyositis and those with skin-predominant dermatomyositis. DESIGN: Retrospective cohort study. SETTING: University hospital outpatient dermatology referral center. Patients Medical records of 91 outpatients with adult-onset dermatomyositis seen between May 26, 2006, and May 25, 2009, were reviewed. MAIN OUTCOME MEASURES: Presence of ILD on thin-slice chest computed tomographic (CT) scans and DLCO. RESULTS: Of the 71 patients with dermatomyositis who had CT or DLCO data, 16 (23%; 95% confidence interval [CI], 13%-33%) had ILD as defined by CT results [corrected]. All patients with ILD had a reduced DLCO, and the ILD prevalence was not different between patients with skin-predominant dermatomyositis (10 of 35 [29% ]) and those with classic dermatomyositis (6 of 36 [17% ]) (P = .27). Eighteen of 71 patients with dermatomyositis (25%; 95% CI, 15%-36%) (7 of 35 [20%] with skin-predominant dermatomyositis; 11 of 36 [31%] with classic dermatomyositis; P = .41) had a low DLCO in the absence of CT findings showing ILD. The prevalence of malignant disease was higher in patients with classic dermatomyositis than in those with skin-predominant dermatomyositis (P = .02), and no patients with skin-predominant dermatomyositis had internal malignant disease. CONCLUSIONS: Radiologic ILD and isolated DLCO reductions, which may signify early ILD or pulmonary hypertension, are common in dermatology outpatients with both classic and skin-predominant dermatomyositis. Because DLCO testing is both inexpensive and sensitive for pulmonary disease, it may be appropriate to screen all patients with dermatomyositis with serial DLCO measurements and base further testing on DLCO results.

Mycophenolate mofetil for interstitial lung disease in dermatomyositis.

OBJECTIVE: To report our experience using mycophenolate mofetil as first-line treatment for dermatomyositis-associated interstitial lung disease. METHODS: We examined the medical records of all 16 dermatomyositis patients with interstitial lung disease seen in our outpatient university hospital dermatology clinic between May 26, 2006, and May 25, 2009. In this retrospective case series, we describe the clinical course of the 4 patients with definitive evidence of interstitial lung disease on radiologic imaging who were treated with mycophenolate mofetil and had pulmonary data available to document their outcome. All of the patients also received prednisone. RESULTS: All 3 patients with at least 1 year of followup receiving mycophenolate mofetil experienced complete normalization of pulmonary function tests (including diffusing capacity for carbon monoxide) and resolution of dyspnea. They were also able to reduce their prednisone doses. The only patient with pre- and posttreatment chest computed tomography imaging had total resolution of her interstitial opacities. The patient with only 5 months of posttreatment followup experienced an improvement in diffusing capacity for carbon monoxide from 44% to 77% predicted, but no change in dyspnea. CONCLUSION: These promising data indicate that mycophenolate mofetil may be a useful therapy for interstitial lung disease in patients with dermatomyositis, but larger studies are needed to more definitively evaluate the role of this medication in therapy.

Spirometry: don't blow it!

Spirometry is a useful test of pulmonary function and can be safely performed in a variety of clinical situations. Although the technique for performing the maneuver is straightforward, there are many sources of variability in results. Specific criteria must be met in order for the test to be considered valid. For the best results, proper instruction and coaching is essential, and patient understanding and effort must be maximized. Appropriate interpretation of spirometry requires several steps, including recognition and reporting of technically sound maneuvers, comparison to an appropriate reference population, and finally application of a well-developed interpretation scheme utilized in the context of patient symptoms and findings. Failure at any point along this path from performance to interpretation can yield misleading results that may ultimately poorly impact patient care. A clear understanding by the provider of proper coding and billing for spirometry is necessary to receive appropriate reimbursement from payers.

Hypoxia altitude simulation test.

A large number of patients with underlying pulmonary disease travel by air each year and are therefore at risk for significant cardiopulmonary effects of induced hypoxia at higher altitudes. The hypoxia altitude simulation test provides a simple way to identify those patients at risk by simulating conditions encountered at high altitude. By asking the patient to breathe a mixture of gases with an oxygen saturation of 15.1%, the test simulates a cabin pressure of 8,000 feet and allows the physician to screen for hypoxia, significant symptoms, and arrhythmias. Repeating the test with supplemental oxygen ensure adequate treatment of those patients who have a decrease in the alveolar pressure of oxygen, significant symptoms, and/or arrhythmias.

Impact of the new ATS/ERS pulmonary function test interpretation guidelines.

RATIONALE: In November 2005, the American Thoracic and European Respiratory Societies jointly published a statement proposing a new interpretation scheme for pulmonary function tests. The practical effect of adoption of these new guidelines has not yet been studied. The purpose of the current study was to address the effects of the new interpretation strategy on the relative distribution of obstructive and restrictive diagnoses in patients evaluated at a single academic medical center laboratory. PATIENTS/METHODS: Pulmonary functions tests from 319 patients were analyzed according to four different interpretation schemes. The number of patients classified according to each as obstructed, restricted, neither, or both were compared, and factors associated with a change in classification using the different approaches were examined. RESULTS: Although similar proportions of patients were identified as restricted using either the "GOLD" scheme (23%) or new approaches (22%), significantly more (P<0.005) were defined as obstructed using the newly proposed scheme (44% versus 33%). Additionally, 36% of subjects defined as obstructed using either the traditional or new schemes were classified differently (i.e., either "gained" or "lost" the diagnosis of obstruction) using the new approach. Women were significantly more likely than men to have a change in classification. CONCLUSIONS: The new interpretation scheme leads to a diagnosis of obstruction in a greater proportion of patients undergoing pulmonary function testing. The clinical significance of this finding has not yet been validated, and its economic impact remains to be assessed.

Lesson learned from ACCESS (A Case Controlled Etiologic Study of Sarcoidosis).

ACCESS (A Case Control Etiologic Study of Sarcoidosis) was funded by the National Institutes of Health and collected data on 704 newly diagnosed, biopsy-proven cases of sarcoidosis and control subjects matched by age, sex, race, and geographic area. The goal of this study was to generate hypotheses about the etiology of sarcoidosis. The major hypothesis of the ACCESS investigators was that sarcoidosis occurs in genetically susceptible individuals through alteration in immune response after exposure to an environmental, occupational, or infectious agent. Strict criteria were used for the diagnosis of sarcoidosis and definitions of specific organ involvement were developed. The patients recruited for ACCESS represent the best clinical description of sarcoidosis at presentation in the United States. The study investigated the following: occupational/environmental triggers using a detailed questionnaire, infectious agents in the blood by polymerase chain reaction of 16s rDNA of microorganisms and cultures for cell wall-deficient mycobacteria, and genetic associations using a questionnaire to determine familial aggregation and candidate gene analysis. No single cause of sarcoidosis was identified. The results of this study are reviewed and possible lessons learned are discussed.

Complications of video-assisted thoracoscopic lung biopsy in patients with interstitial lung disease.

BACKGROUND: Current guidelines recommend surgical lung biopsy for diagnosis of interstitial lung diseases (ILDs) in selected patients. To shed light on the risk-benefit ratio for this recommendation, we examined the morbidity and mortality associated with video-assisted thoracoscopic surgical (VATS) lung biopsy in a group of outpatients. METHODS: A retrospective cohort study was conducted of 68 consecutive ambulatory patients with radiographically apparent interstitial lung disease (ILD) referred for VATS biopsy during a 6-year period. Incidence of postoperative mortality, prolonged air leaks, pneumonias, and re-admissions were calculated. Risk factors for complications of surgery were examined. RESULTS: Three deaths occurred within 60 days after biopsy for a mortality rate of 4.4% (95% confidence interval [CI], 1% to 12%), and 19.1% (95% CI, 11% to 31%) experienced one or more complications of surgery. Risk factors for morbidity included preoperative dependence on oxygen therapy and pulmonary hypertension. The three patients who died had usual interstitial pneumonia on their biopsy specimen and were reintubated postoperatively for acute lung injury. Aggregation of articles published over the past 10 years reporting on surgical lung biopsy for the diagnosis of ILD yielded a postoperative mortality rate of 2% to 4.5%. CONCLUSIONS: VATS lung biopsy for diagnosis of ILD, even in ambulatory patients, is not an entirely benign procedure. Biopsy rarely may trigger an acute exacerbation of usual interstitial pneumonitis. The risk of postoperative complications appears to be greatest in those dependent on oxygen and those who have pulmonary hypertension. This information may be used in weighing the risk-benefit ratio of biopsy in individual patients.

Relationship of environmental exposures to the clinical phenotype of sarcoidosis.

STUDY OBJECTIVES: Sarcoidosis is a granulomatous disorder with heterogeneous clinical manifestations, which are potentially reflective of a syndrome with different etiologies leading to similar histologic findings. We examined the relationship between environmental and occupational exposures, and the clinical phenotype of sarcoidosis. DESIGN: We performed a cross-sectional study of incident sarcoidosis cases that had been identified by A Case Control Etiologic Study of Sarcoidosis. Subjects were categorized into the following two groups: (1) pulmonary-only disease; and (2) systemic disease (with or without pulmonary involvement). Logistic regression was used to examine the associations of candidate exposures with clinical phenotype. SETTING: Ten academic medical centers across the United States. PATIENTS: The current study included 718 subjects in whom sarcoidosis had been diagnosed within 6 months of study enrollment. Patients met the following criteria prior to enrollment: (1) tissue confirmation of noncaseating granulomas on tissue biopsy on one or more organs within 6 months of study enrollment with negative stains for acid-fast bacilli and fungus; (2) clinical signs or symptoms that were consistent with sarcoidosis; (3) no other obvious explanation for the granulomatous disease; and (4) age > 18 years. MEASUREMENTS AND RESULTS: Several exposures were associated with significantly less likelihood of having extrapulmonary disease in multivariate analysis, including agricultural organic dusts and wood burning. The effects of many of these exposures were significantly different in patients of different self-defined race. CONCLUSIONS: The differentiation of sarcoidosis subjects on the basis of clinical phenotypes suggests that these subgroups may have unique environmental exposure associations. Self-defined race may play a role in the determination of the effect of certain exposures on disease phenotypes.

Bronchoscopy for atelectasis in the ICU: a case report and review of the literature.

Fiberoptic bronchoscopy has become a commonplace procedure in ICUs. Despite the fact that one of the most common indications for bronchoscopy is the presence of retained secretions and atelectasis, there is little research dedicated to its safety and utility in this clinical situation. This article presents a case of an intubated trauma victim who had undergone numerous bronchoscopic procedures, with varying degrees of success, for retained secretions and atelectasis. This review then seeks to answer the following three main questions regarding bronchoscopy in critically ill patients: (1) Is bronchoscopy effective in resolving atelectasis? (2) Is bronchoscopy superior to other means of resolving atelectasis? (3) Is bronchoscopy safe in critically ill patients? The patient was a 28-year-old man with no significant medical history who presented to the emergency department after his car was hit by a dump truck. He was found to have multiple leg fractures and a splenic rupture, and he was taken to the operating room for an exploratory laparotomy, splenectomy, and reduction of his fractures. He was then brought to the surgical ICU intubated, sedated, and receiving mechanical ventilation. Over the next 6 h, he developed progressive hypoxemia and diffuse, bilateral alveolar infiltrates on a chest radiograph (CXR). Four days postoperatively, a routine CXR revealed total atelectasis of his right upper lobe (RUL). Emergent bronchoscopy was performed, and a large mucus plug obscuring the RUL bronchus was removed. Follow-up CXR demonstrated resolution of the atelectasis. The next day, RUL atelectasis was again seen on his CXR. A repeat bronchoscopic examination and BAL failed to reveal any plug. A follow-up CXR showed continued atelectasis. Over the next week, the patient underwent daily bronchoscopy for atelectasis with variable degrees of improvement. Over the next 3 weeks, his pulmonary status improved until he was eventually extubated, and 1 month after hospital admission he was discharged to rehabilitation.

Is chronic beryllium disease sarcoidosis of known etiology?

Sarcoidosis is a granulomatosis that predominantly affects the lungs and is of unknown etiology. Chronic beryllium disease is a granulomatosis that predominantly affects the lungs and is due to a hypersensitivity response to beryllium. Similarities and differences between these two disorders are reviewed and lessons for both sarcoidosis and chronic beryllium disease are discussed.