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Pleural and pericardial involvements are well recognized in eosinophilic granulomatosis with polyangiitis (EGPA) but considered rare manifestations of the other forms of antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV).What are the frequency and clinical characteristics of pleuritis and pericarditis in AAV?and Methods: Using an institutional database of 1,830 patients with AAV, we analyzed clinical notes and diagnosis codes for key words related to pleuritis and pericarditis. Chart review to confirm these findings was performed.Eighty-eight of 1,058 patients (8.3%) with granulomatosis with polyangiitis (GPA), 27 of 267 (10.1%) with microscopic polyangiitis (MPA), and 35 of 201 (17.4%) with EGPA had a manifestation of pleuritis and/or pericarditis attributable to vasculitis. There was a higher frequency of pericarditis in EGPA compared with that in the other AAVs (P .01). There was no difference in the frequency of pleuritis in GPA, MPA, or EGPA. In the 156 patients with AAV with pleuritis and/or pericarditis, this was a presenting feature in 127 (81.4%). Overall, it was a presenting feature in 6.9% of all patients with AAV, including 6.5% with GPA, 8.6% with MPA, and 15.9% with EGPA.Pleuritis and pericarditis occur across all the AAVs and, when present, are commonly presenting features of these diseases. Patients with EGPA have a higher proportion of pericardial involvement compared with pleural involvement, whereas this distribution is more equal in patients with GPA and MPA. Pleuritis and pericarditis are underrecognized features of AAV. All forms of AAV should be considered in the differential diagnosis when evaluating a patient with pleuritis or pericarditis.

A 39-year-old white woman with a history of adult-onset asthma, chronic sinusitis, and nasal polyposis presented to the ED with dyspnea and left lower extremity weakness and pain. Three months prior to her presentation she had an uncomplicated delivery of her second child, but during her pregnancy she experienced increasing asthma symptoms and nasal congestion. These symptoms progressed after delivery despite treatment with albuterol inhalers and antibiotics.

Case Presentation A 56-year-old man was admitted to the ICU with chest pain, cough, hemoptysis, increasing dyspnea, and orthopnea for 1 week. The patient reported an 8-kg weight loss over the last month and recurrent wheezing episodes for approximately 1 year. He had a history of tobacco smoking and excessive alcohol consumption, both of which he stopped 15 years ago. His medical history included high BP treated with amlodipine and an episode of drug-induced angioedema 8 years ago. He had no history of recent travel.

Case Presentation A 56-year-old man with a history of common variable immunodeficiency (CVID), in addition to recurrent bronchitis and pneumonia, presented with progressively worsening shortness of breath over a period of a few months. He was able to conduct his activities of daily living at baseline; however, his condition declined over a period of months to the point of shortness of breath with climbing a flight of stairs. The patient also developed a frequent dry cough and wheezing. He denied fever, chest pain, weight loss, and hemoptysis. He had been diagnosed with CVID in 1968, at 7 years of age, after recurrent episodes of bronchitis and pneumonia, and was treated with IV immunoglobulin monthly for decades. The patient was a lifelong nonsmoker and had no significant environmental or occupational exposures. He was referred to our hospital for further management.

A 62-year-old white woman was admitted with shortness of breath, wheezing, and cough. While in the hospital a generalized pruritic skin rash developed on her trunk and upper and lower extremities. She did not have any fevers, chills, or night sweats. The patient was known to have chronic, difficult-to-control asthma despite being compliant with a treatment regimen consisting of inhaled albuterol, high-dose inhaled steroids, salmeterol, and montelukast. Her medical history was significant for hypertension and gout. She had no family history of asthma. The patient was a life-long nonsmoker and did not drink alcohol. During this hospitalization, she was started on prednisone 40 mg/d po in addition to her home medications.

The term vasculitis encompasses a number of distinct clinicopathologic disease entities, each of which is characterized pathologically by cellular inflammation and destruction of the blood vessel wall, and clinically by the types and locations of the affected vessels. While multiple classification schemes have been proposed to categorize and simplify the approach to these diseases, ultimately their diagnosis rests on the identification of particular patterns of clinical, radiologic, laboratory, and pathologic features. While lung involvement is most commonly seen with the primary idiopathic, small-vessel or antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides of Wegener granulomatosis, microscopic polyangiitis, and Churg-Strauss syndrome, one should remember that medium-vessel vasculitis (ie, classic polyarteritis nodosa), large-vessel vasculitis (ie, Takayasu arteritis), primary immune complex-mediated vasculitis (ie, Goodpasture syndrome), and secondary vasculitis (ie, systemic lupus erythematosus) can all affect the lung. However, for the purpose of this review, we will focus on the ANCA-associated vasculitides.

Study objectives We previously reported eight patients who developed Churg-Strauss syndrome in association with zafirlukast treatment for asthma and postulated that the syndrome resulted from unmasking of a previously existing condition due to corticosteroid withdrawal and not from a direct drug effect. The availability of montelukast, a new leukotriene receptor antagonist with a different molecular structure, permitted us to test this hypothesis. Our goals were to ascertain whether the Churg-Strauss syndrome developed in patients taking montelukast and other novel asthma medications, and to describe potential mechanisms for the syndrome. Design Case series. Setting Outpatient and hospital practices of pulmonologists in the United States and Belgium. Patients Four adults (one man, three women) who received montelukast as treatment for asthma; two women who received salmeterol/fluticasone therapy, but not montelukast. Results Churg-Strauss syndrome developed in the four asthmatic patients who received montelukast. In each case, there was a long history of difficult-to-control asthma characterized by multiple exacerbations that had required frequent courses of oral systemic corticosteroids or high doses of inhaled corticosteroids for control. Two other asthmatics who received fluticasone and salmeterol but not montelukast therapy developed the same syndrome with tapering doses of oral or high doses of inhaled corticosteroids. Conclusions The occurrence of Churg-Strauss syndrome in asthmatic patients receiving leukotriene modifiers appears to be related to unmasking of an underlying vasculitic syndrome that is initially clinically recognized as moderate to severe asthma and treated with corticosteroids. Montelukast does not appear to directly cause the syndrome in these patients.

Churg-Strauss syndrome (CSS) is an uncommon systemic vasculitis with an increase in the number of eosinophils in the peripheral blood and tissues. Its pathogenesis is unknown, and there is no evidence that genetic factors influence susceptibility to this disease. We present a case of familial CSS in two sisters with atopic-type bronchial asthma and negative perinuclear anti-neutrophil cytoplasmic antibody results. We investigated the human leukocyte antigen typing of the sisters and their six living siblings but found no evidence for heritability of CSS. To our knowledge, this is the first report of familial CSS.

We report 4 cases of Churg-Strauss syndrome (CSS) that occurred in patients being treated with corticosteroids for a diagnosis of asthma. One patient had asthma, eosinophilia, and eosinophilic lymphadenopathy that regressed with higher doses of corticosteroids. The second patient had both eosinophilic tissue infiltration and symptoms suggestive of vasculitis, while the remaining two patients had overt vasculitis; in all three, vasculitis developed after tapering or discontinuation of corticosteroid therapy. Two patients died of their disease. We have labelled these cases as formes frustes CSS. Our observations suggest that some cases of CSS may be partially or totally suppressed by corticosteroid therapy for asthma for very long periods and that asthmatic subjects maintained on low-dose corticosteroid therapy or asthmatic subjects whose corticosteroid doses are being tapered should be carefully monitored for the development of CSS signs and symptoms. (CHEST 1995; 108:320-23)

Background Churg-Strauss syndrome (CSS) is a rare systemic vasculitis associated with asthma, eosinophilia, sinusitis, and pulmonary infiltrates. CSS has been reported in association with asthma therapies. Materials and methods The objective is to describe the characteristics of CSS in patients treated with the anti-IgE antibody omalizumab (Xolair; Genentech Inc; South San Francisco, CA). A retrospective review of available data to identify cases of CSS was performed using the Novartis Argus global drug safety database for omalizumab in asthma patients. Results We identified 34 potential cases of CSS. Of these, 13 cases fulfilled at least four of the six criteria identified in the American College of Rheumatology classification criteria. Eight of the patients in these 13 definite or probable cases (62%) had CSS symptoms prior to receiving omalizumab or described symptom onset just after corticosteroid weaning. Six of the 13 patients (46%) were confirmed as having been treated with corticosteroids for what was perceived to be severe asthma; when corticosteroids were tapered in conjunction with omalizumab treatment, CSS symptoms appeared just after the tapering. There were 4 other cases of possible CSS, and the remaining 17 patients were judged to not have CSS. Conclusions CSS may develop in patients receiving asthma medications who have an underlying eosinophilic disorder that is unmasked by the withdrawal of therapy with corticosteroids, or in patients who delay therapy in favor of other medications. Omalizumab treatment may unmask CSS due to the weaning of corticosteroids in some asthma patients or may delay corticosteroid treatment allowing for CSS to manifest. Trial registration ClinicalTrials.gov Identifier: NCT00252135

We report the first case of pulmonary Corynebacterium ulcerans infection mimicking Churg-Strauss syndrome (CSS). Productive cough, fever, general fatigue, and weight loss developed in a 50-year-old man. Laboratory data revealed prominent eosinophilia and elevated serum IgE. On chest images, multiple nodules and cavities were predominantly detected in the right lung. Histopathologic examination showed necrotizing granulomas and vasculitis with massive eosinophilic infiltration identical to the findings seen in CSS; however, clusters of Gram-positive, coryneform rods were observed in the alveolar spaces. A toxigenic strain of C ulcerans was isolated from lung tissue. The patient was treated with antibiotics, and a favorable clinical course ensued.

Purpose This study aims to describe the clinical course and prognostic factors of patients with small-vessel vasculitis admitted to a medical ICU. Methods We reviewed the clinical records of 38 patients with small-vessel vasculitis admitted consecutively to the ICU between January 1997 and May 2004. The APACHE (acute physiology and chronic health evaluation) III prognostic system was used to determine the severity of illness on the first ICU day; the sequential organ failure assessment (SOFA) score was used to measure organ dysfunction, and the Birmingham vasculitis activity score for Wegener granulomatosis (BVAS/WG) was used to assess vasculitis activity. Outcome measures were the 28-day mortality and ICU length of stay. Results Nineteen patients (50%) had Wegener granulomatosis, 16 patients (42%) had microscopic polyangiitis, 2 patients had CNS vasculitis, and 1 patient had Churg-Strauss syndrome. Reasons for ICU admission included alveolar hemorrhage in 14 patients (37%), sepsis in 5 patients (13%), seizures in 3 patients (8%), and pneumonia in 2 patients (5%). The median ICU length of stay was 4.0 days (interquartile range, 2.0 to 6.0 days). The APACHE III score was lower in survivors than nonsurvivors (p = 0.010). The predicted hospital mortality was 54% for nonsurvivors and 21% for survivors (p = 0.0038). The mean SOFA score was 11.6 (SD, 2.6) in nonsurvivors, compared to 6.9 (SD, 2.4) in survivors (p = 0.0004). Mean BVAS/WG scores were 8.6 (SD, 3.6) in nonsurvivors and 4.7 (SD, 4.6) in survivors (p = 0.0889). Twenty-six percent of the patients received invasive mechanical ventilation, and 33% underwent dialysis. The 28-day and 1-year mortality rates were 11% and 29%, respectively. Conclusions The mortality of patients with small-vessel vasculitis admitted to the ICU is lower than predicted, and alveolar hemorrhage is the most common reason for ICU admission.

Churg-Strauss syndrome (CSS) is a rare form of systemic vasculitis occurring in patients with asthma and hypereosinophilia. For optimal treatment, prompt distinction of CSS from asthma is necessary; however, there are few serologic screening markers for this purpose. Vascular endothelial growth factor (VEGF), a vascular permeability factor, has been associated with other systemic vasculitis such as Wegener granulomatosis and giant-cell arteritis.The aim of this study was to clarify the clinical value of the measurement of serum VEGF for the distinction of CSS from asthma.We investigated serum VEGF levels in 18 CSS patients, 19 asthma patients, and 12 acute bronchitis patients. We also performed immunohistochemical analysis for VEGF.The serum VEGF levels of CSS patients were significantly higher than those of asthma patients and acute bronchitis patients. The sensitivity and specificity to distinguish CSS from asthma were 93.3% and 81.8%, respectively (cutoff, 600 pg/mL). Infiltrating eosinophils stained intensely positive for VEGF, and serum VEGF levels showed a significant correlation with peripheral eosinophil counts. Serum VEGF levels decreased significantly after therapy (p0.001). The infiltrating eosinophils in the CSS lesion stained positive for VEGF in the immunohistochemical analysis.VEGF is one of the useful screening markers for the distinction of CSS from asthma. We suggest that VEGF might be associated with the pathogenesis of CSS.