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Churg-Strauss syndrome

Churg-Strauss syndrome (also known as allergic granulomatosis) is a form of vasculitis due to autoimmunity, leading to necrosis. It involves mainly the arteries, the lungs (it begins as a severe type of asthma), and nerves. Churg-Strauss syndrome is classified both as a type of polyarteritis nodosa and, more broadly, as a diffuse connective tissue disease.


Diagnostic markers include eosinophil granulocytes and granulomas in affected tissue and pANCA antibodies against neutrophil granulocytes. Differentiation from Wegener's granulomatosis can be difficult.

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Churg-Strauss syndrome associated with zafirlukast
From CHEST, 7/1/98 by daren L. Knoell

The leukotriene receptor antagonist zafirlukast (Accolate; Zeneca Pharmaceuticals; Wilmington, Del) recently was approved for use as maintenance therapy for persistent asthma. This new product has been well received due to convenient dosing and relatively few side effects. Based on initial success with this product, it is likely that similar compounds will be available for use in the near future. In this report, a case is described of a 47-year-old white man with moderate persistent asthma in whom Churg-Strauss syndrome developed while he was receiving zafirlukast therapy. Acute respiratory insufficiency, arthralgia, and prominent rash developed which required hospitalization. The patient's symptoms rapidly reversed following discontinuation of zafirlukast therapy and administration of systemic corticosteroids. Although the incidence of Churg-Strauss syndrome associated with zafirlukast therapy is rare, this case report illustrates steps that may be taken to diagnose quickly and treat this life-threatening condition should it occur.

(CHEST 1998; 114:332-334)

Key words: asthma; eosinophilic lung disease; lenkotriene; vasculitis; zafirlukast

In recent years, an increased understanding of the pathophysiology of asthma has identified a complex network of interactions among a variety of airway cells and the inflammatory mediators, including the leukotrienes, that they release.[1] Antileukotriene therapy for asthma represents a new class of therapeutic compounds used to control inflammation and bronchoconstriction in the airways of asthmatic patients.[2] Zafirlukast (Accolate; Zeneca Pharmaceuticals; Wilmington, Del) is the first new medication in this class to be approved for use in asthmatic patients. Zafirlukast is an [LTD.sub.4]-receptor antagonist that blocks the effects of the cysteinyl leukotrienes ([LTC.sub.4], [LTD.sub.4], and [LTE.sub.4]) on targeted cells such as smooth muscle and mucus cells.[2] Zafirlukast is generally well tolerated and can be conveniently administered as an oral tablet twice daily taken on an empty stomach. With the exception of significant but preventable drug-drug interactions, has been touted as a safe and effective medication for the prophylaxis and long-term treatment of asthma. In this case report, the development of acute respiratory insufficiency and hypereosinophilic syndrome is described in a 47-year-old male asthmatic patient who was receiving zafirlukast therapy.


A 47-year-old white man with a 3-year history of moderate persistent asthma was self-referred to the outpatient asthma clinic of Ohio State University School of Medicine for further evaluation of steroid-dependent asthma. Evaluation at the time included an eosinophil count of 71, an IgE level of 50, negative results of Aspergillus immunodiffusion serologic tests, a normal chest x-ray film, and a normal high-resolution chest CT scan. Over the next year, the patient experienced multiple asthma exacerbations that required treatment with prednisone. Videolaryngostroboscopy showed no evidence of vocal cord dysfunction, and another IgE level remained within normal limits. A sinus CT scan showed pansinusitis and nasal polyps. Following surgery for nasal polyp excision, the patient was noted to have a marked improvement in his asthma symptoms and no longer required oral prednisone therapy. At this time, the patient received maintenance therapy with a regimen consisting of fluticasone propionate (Flovent; GlaxoWellcome; Research Triangle Park, NC), salmeterol xinafoate (Serevent Inhalation Aerosol; Glaxo Wellcome; Research Triangle Park, NC), and an albuterol inhaler (Proventil; Schering; Kenilworth, NJ) which he rarely required.

Approximately 2 months later, the patient noted an increase in asthmatic symptoms secondary to an upper respiratory tract infection. A decision was made to initiate a 1-week trial of zafirlukast 20-mg tablets twice a day. Two months later, the patient reported significant improvement in asthmatic symptoms. The patient had not taken zafirlukast since his sample had run out. The patient stated that zafirlukast had contributed mildly to his improvement. The patient also noted no adverse events related to zafirlukast use. Two months later, the patient returned to the asthma clinic due to increased asthmatic symptoms. Flunisolide (Aerobid; Forest Pharmaceuticals; New York) had recently been substituted for fluticasone. Subsequently, the patient noted an increase in asthmatic symptoms. A decision was made to reinitiate zafirlukast therapy at 20 mg twice daily based on the favorable response which had been previously attained.

Within 1 month, the patient experienced a progressive rash over his trunk and extremities, arthralgia, sinus congestion, malaise, cough, and a fever lip to 38.8 [degrees] C. He was originally treated as an outpatient with doxycycline and nonsteroidal antiinflammatory drugs but required hospital admission when his symptoms progressed. The skin eruption was noted over his {ace, arms, and trunk and was pruritic and painful. The chest x-ray film demonstrated basilar reticulonodular infiltrates (Fig 1), and laboratory findings showed a blood eosinophil count of 4,350 and a positive titer for rheumatoid factors.


The patient was transferred the following day to Ohio State University Hospitals for possible pneumonia and skin rash. On physical examination, there were scattered coarse crackles in the bases and diffuse inspiratory wheezes and squeaks. Examination of the extremities and skin revealed a maculopapular rash involving the scalp, the upper area of the back, and both forearms, with some vesicular violaceous lesions in addition to erythematous papular lesions. There were also small purpuric lesions on the dorsum of both feet and ankles. There were swelling, pain, and decreased range of motion of his right shoulder and both ankles. In addition, the total IgE level was elevated (851 IU/L) and a nentrophil cytoplasmic antibody test was negative. Therapy was begun with IV hydrocortisone, acyclovir, ceftriaxone disodium, and erythromycin; other medications, including zafirlukast, were discontinued. A skin biopsy showed microvascular injury and eosinophilia. Subsequently, the patient underwent fiberoptic bronchoscopy with transbronchial biopsy, endobronchial biopsy, and BAL. The bronchial mucosa was diffusely edematous with associated petechial-appearing mucosal lesions consistent with vasculitis. A biopsy specimen of tissue was notable for interstitial eosinophils, eosinophil-containing granulomas, and patchy areas of small blood vessel vasculitis with numerous eosinophils in the vessel walls (Fig 2). BAL fluid consisted of macrophages (24%), lymphocytes (24%), neutrophils (44%), and eosinophils (8%). A bilateral lower extremity Doppler study and a cardiac echocardiogram showed no abnormalities. BAL fluid cultures were negative for bacteria, mycobacteria, Legionella, and fungi, and skin tissue viral cultures were negative for viruses. A urine test for Legionella and serum Mycoplasma IgM antibodies was negative. There was late growth of parainfluenza virus from the BAL fluid. As a result, all intravenous antibiotics were discontinued during the hospital stay.


Following initiation of intravenous corticosteroids, the patient had immediate improvement in his arthralgia, rash, and shortness of breath. He remained afebrile throughout his inpatient stay. In addition, blood eosinophil counts quickly returned to normal, and the patient was ambulating with minimal dyspnea on day 3 after admission. On the 4th hospital day, the patient was discharged with medications including a flunisolide inhaler, an albuterol inhaler to be used as needed, cefuroxime tablets, and prednisone tablets, 60 mg every morning. Over the next 5 months, the patient was weaned from prednisone therapy, but within 1 week of discontinuing steroids, recurrent wheezing, a reduction in peak expiratory flow rates, an eosinophil count of 1,000, and recurrence of the basilar reticulonodular pulmonary infiltrates developed. Once again, his symptoms resolved promptly with resumption of prednisone therapy.


Allergic angiitis and granulomatosis was originally described by Churg and Strauss[3] in 1951. Patients presenting with Churg-Strauss syndrome usually have a history of allergic disease for 8 to 10 years before presentation; all patients have asthma or a history of asthma and most have allergic rhinitis.[4] A number of organs can be involved in Churg-Strauss syndrome. Upper airway findings can include sinusitis, rhinitis, and nasal polyps. Skin findings are found in 70% of patients and may include nodules, purpura, or urticaria. Arthralgia and myalgia occur in approximately one half of all patients, and most patients present with fever. The majority of patients will present with pulmonary infiltrates on a chest x-ray film, elevation of serum IgE levels, and peripheral eosinophilia.[5] Approximately 50% of patients will have an abnormal antineutrophil cytoplasmic antibody level, usually in the pANCA form. Open-lung biopsy remains the diagnostic "gold standard."[6] Histologic findings usually demonstrate a necrotizing giant cell vasculitis particularly in the small arteries and veins. Eosinophils often are present and accumulate in blood vessels, interstitial tissue, and alveolar structures.[4] Corticosteroids are the treatment of choice for patients with Churg-Strauss syndrome. Most patients respond favorably with a mean survival of 9 years compared with that of patients who do not receive treatment anti have a mean survival of approximately 3 months.

As just described, the patient whose case is presented in this report initially exhibited many of the features present in Churg-Strauss syndrome. It is unlikely that doxycycline contributed to the patient's symptoms since the medication was started well after the beginning of symptoms. Although nonsteroidal anti-inflammatory agents have been implicated in eosinophilic lung disease, likewise, they are unlikely the cause since this medication was initiated after the onset of systemic symptoms. Although this patient grew parainfluenza virus from the BAL fluid, this common respiratory virus has not been reported to cause eosinophilic vasculitis although it can cause exacerbations of asthma.

The temporal relationship between use of zafirlukast anti the development of systemic vasculitis suggests a possible causal relationship. The severity of the illness precluded a rechallenge of the medication, however. The manufacturers of zafirlukast currently are investigating seven cases in which this product has been implicated as a cause of Churg-Strauss syndrome (personal communication; M. E. Turner, MD; Zeneca Pharmaceuticals; October 9, 1997). Interestingly, the seven case reports of Churg-Strauss syndrome associated with zafirlukast therapy were complicated by the concomitant weaning of oral corticosteroids. Because Churg-Strauss syndrome typically arises in the setting of difficult to control asthma and because leukotriene inhibitors often are used as "steroid sparing" agents in patients with severe asthma, it is possible that in at least some of these cases zafirlukast unmasked Churg-Strauss syndrome previously made occult by long-term steroid use. In the patient reported here, Churg-Strauss syndrome developed while he was receiving zafirlukast; he had not taken prednisone for approximately 6 months prior to experiencing untoward events. A recent case series[7] of eight adult patients reported similar symptoms while patients were taking zafirlukast and undergoing corticosteroid withdrawal. Because of the low incidence and uncertain causal relationship between Churg-Strauss syndrome and zafirlukast, the manufacturer and the US Food and Drug Administration do not currently recommend that patients using zafirlukast discontinue use if they do not have symptoms of vasculitis.

It is important that health care providers and patients using zafirlukast and similar products be aware of the early symptoms of drug-associated Churg-Strauss syndrome to prevent life-threatening adverse events from occurring. For patients in whom Churg-Strauss syndrome develops, discontinuation of zafirlukast seems prudent, and treatment with corticosteroids can be life-saving.


[1] Barnes PJ. Frontiers in medicine: new aspects in asthma. J Intern Med 1992; 231:453-61

[2] Spector S. Leukotriene inhibitors and antagonists in asthma. Ann Allergy Asthma Immunol 1995; 75:463-73

[3] Churg J, Strauss L. Allergic granulomatosis, allergic angiitis and peri-arteritis nodosa. Am J Pathol 1951; 27:277-301

[4] Chumbley LC, Harrison EG, DeRemee RA. Allergic granulomatosis and angiitis (Churg-Strauss syndrome): report and analysis of 30 cases. Mayo Clin Proc 1977; 52:477-84

[5] Lanham JG, Elkon KB, Pusey CD, et al. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome. Medicine 1984; 63:65-81

[6] Allen JN, Davis WB. Eosinophilic lung disease. Am J Respir Crit Care Med 1994; 150:1423-38

[7] Wechsler ME, Garpestad E, Flier SR, et al. Pulmonary infiltrates, eosinophilia, and cardiomyopathy following corticosteroid withdrawal in patients with asthma receiving zafirlukast. JAMA 1998; 279:455-57

(*) From the Divisions of Pharmacy (Dr. Knoell, Pathology (Dr. Lucas), and Pulmonary and Critical Care Medicine (Dr. Allen), Ohio State University School of Medicine, Columbus.

Manuscript received October 24, 1997; revision accepted December 19, 1997.

COPYRIGHT 1998 American College of Chest Physicians
COPYRIGHT 2000 Gale Group

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