eMedicine Specialties > Neurology > Pediatric Neurology

Churg-Strauss Disease

Author: Robert Rust Jr, MD, Thomas E Worrell Jr Professor of Epileptology and Neurology, Co-Director of FE Dreifuss Child Neurology and Epilepsy Clinics, University of Virginia School; Clinical and Residency Training, Child Neurology, University of Virginia Hospital and Clinics
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Updated: Sep 28, 2006

Introduction

Background

Churg-Strauss disease is one of the 3 important common leukocytoclastic (ie, fibrinoid, necrotizing, inflammatory) systemic small-vessel vasculitides associated with antineutrophil cytoplasm antibodies (ANCAs). Of these, it is the least common. The others are Wegener granulomatosis (WG) and microscopic polyangiitis (MPA). Although these conditions are not thought to be directly infectious, microbial superantigens may play a role in provoking the onset of the dysregulated immune response that gives rise to these conditions.

In 1951, Jacob Churg and Lotte Strauss of Sweden first made a distinction between Churg-Strauss disease and polyarteritis nodosa (PAN). The Chapel Hill Consensus classification of systemic vasculitides distinguished PAN from Churg-Strauss because PAN predominantly affects medium-sized blood vessels (medium sized and small arteries), while Churg-Strauss predominantly affects small vessels (capillaries, venules, arterioles). The medium-sized vessel predilection of PAN is shared with Kawasaki disease, conditions that spare capillaries, arterioles, and venules. Lie (1994) has emphasized the fact that Churg-Strauss, WG, and MPA are not exclusively small-vessel vasculitides but may also involve the medium-sized vessels that are the characteristic targets of PAN.

In addition to the ANCA-associated small-vessel vasculitides WG and MPA, there are also non-ANCA immune complex associated small-vessel vasculitides such as Schönlein-Henoch purpura (SHP), essential cryoglobulinemic vasculitis (ECV), lupus vasculitis, serum sickness vasculitis, and infection-induced immune complex vasculitis. Churg-Strauss is distinguished from these not only by the presence of ANCA but as well by its association with asthma and by the characteristically associated tissue and blood eosinophilia.

Churg-Strauss disease invariably involves the lungs and may, in addition, affect a wide variety of other tissues and organs of the body, including tissues of the nervous system. However, no single known manifestation of Churg-Strauss disease is pathognomonic. All of the clinical features overlap in some degree with manifestations found in other systemic necrotizing vasculitides.

In most cases of Churg-Strauss disease, the combination of predominantly respiratory tract disease, in association with a characteristic disease evolution involving asthma and eosinophilia is highly suggestive and the diagnosis may usually readily be confirmed by the identification of ANCA-positivity and demonstration of the distinctive eosinophilic vasculitis of small and some medium-sized vessels.

Nonetheless, it should be noted that occasional examples of diseases that straddle the boundaries between PAN, WG, MPA, and Churg-Strauss disease are encountered for which it is difficult with certainty which label is most appropriate. For this reason, a recent classification system by Watts and Scott combined these particular entities under the general heading primary systemic vasculitides.

Churg-Strauss disease also has considerable overlap with idiopathic hypereosinophilic syndrome. In such instances, the distinction of Churg-Strauss from these various other conditions, particularly those that share ANCA positivity, is usually made on the basis of prodromal asthma indicative of Churg-Strauss disease. In instances where the kidney is involved, ANCA-positive vasculitides also share, in distinction to other small-vessel vasculitides, presence of pauci-immune crescentic glomerulonephritis.

Idiopathic hypereosinophilic syndrome (IHES) closely resembles Churg-Strauss disease, differing particularly in that the prodromal asthmatic phase is absent. IHES is defined by (1) peripheral eosinophil count greater than 1500/µL for at least 6 months, (2) evidence of characteristic organ involvement, and (3) absence of other known cause. It affects men aged 20-50 years. The initial manifestations are cardiac, consisting of eosinophilic myocarditis with endocardial damage and confirmed by endomyocardial biopsy. Restrictive cardiomyopathy may ensue as thrombosis develops, promoted by the damaged endocardial surface. Fibrosis then occurs. Mitral or tricuspid incompetence may develop as a result of fibrotic degeneration of the chordae tendineae. In some instances, dilated cardiomyopathy develops.

Cardioembolic disease in IHES may produce neurological manifestations, but a primary encephalopathy also has been described. Sensory or mixed sensorimotor peripheral polyneuropathy, closely resembling that found in Churg-Strauss disease, may develop in a symmetrical or asymmetrical distribution. Mononeuropathy multiplex also may develop. Although prodromal asthma is not a feature, as many as 40% of patients with IHES manifest a persistent, dry cough at some stage of illness. Pulmonary dysfunction also may result from cardiac failure or cardiogenic pulmonary embolism.

Pulmonary infiltrates may be found in as many as 30% of cases of IHES, tending not to be peripheral. Parenchymal eosinophilic infiltration may be found and, rarely, eosinophilic pleural effusions develop. Diarrhea complicates about 20% of cases, while eosinophilic gastritis, enteritis, or colitis occasionally has been reported. Dermatologic manifestations may include urticaria, angioedema, or erythematous pruritic papules and nodules. Raynaud phenomenon, arthralgias, or joint effusions are occasional complications.

Pathophysiology

Studies of affected peripheral nerve tissues show that once the stage of epineural necrotic vasculitis has been achieved, activated cytotoxic T-lymphocyte clones (ie, cluster of differentiation 8 [CD8⁺] suppressor/cytotoxic and CD4⁺ helper cells) begin to outnumber eosinophils and predominate in the inflammatory exudate. Occasionally, CD20⁺ B lymphocytes are found in the inflammatory exudate, and deposits of immunoglobulin (IgG), immunoglobulin E (IgE), and C3d antibodies may be detected.

Cytokines undoubtedly participate in this autoimmune process. Patients with Churg-Strauss disease have markedly increased serum levels of interferon alpha and interleukin (IL)–2 and moderate increases of TNF-alpha and IL-1beta similar to those observed in PAN. Elevations of serum IL-6 concentrations have been shown to precede the rise in serum rheumatoid factors that may accompany the onset of an exacerbation of Churg-Strauss vasculitis. Thus, the IL-6 may be an important triggering factor. The rheumatoid factors are chiefly of IgG and immunoglobulin M (IgM) classes, rather than immunoglobulin A (IgA) or IgE.

Perinuclear ANCAs (p-ANCAs), directed against myeloperoxidase, are found in patients with Churg-Strauss disease. Antibodies with this pattern of staining and antigenic specificity also are found in the uncertainly classified entity microscopic polyangiitis. Recent work suggests that a mutation in exon 11 of the CD18 gene may be permissive of the elaboration of antimyeloperoxidase antibodies in either of these 2 conditions.

In Wegener granulomatosis, on the other hand, the presence of cytoplasmic ANCAs (c-ANCAs) with antigenic specificity for proteinase-3 is characteristic. A case of one patient with p-ANCAs directed against myeloperoxidase and c-ANCAs against proteinase-3 has been reported; the patient manifested a combination of clinical characteristics suggesting both Churg-Strauss disease and temporal arteritis.

These immunological disturbances may provoke the translocation of proteinase-3 from within the azurophilic granules of PMN cells to the surface of the cell membrane. The attachment of PMN cells to the endothelial surface likely is enhanced by cytokine-mediated induction of adhesion molecules (eg, lymphocyte function-associated protein 1, IL adhesion molecule 1, endothelial-leukocyte adhesion molecule 1). Antiendothelial cell antibodies, detectable in many different primary vasculitic conditions as well as in systemic autoimmune disease with a vasculitic component, also may play a role in Churg-Strauss disease.

Abnormal expression of CD95 cellular receptors (producing a soluble splice variant rather than the usual membrane-bound isoform) may participate in the pathogenesis of Churg-Strauss disease. The result is impairment of normal apoptotic processes whereby lymphocytes and eosinophils are eliminated. This permits abnormal oligoclonal expansion of specific T-cell clones, which may mediate cellular injury. The soluble CD95 isoform has been termed eosinophil survival factor.

Epidemiological association of Churg-Strauss with the utilization of antileukotrienes for the treatment of asthma suggested the possibility that, in some instances, Churg-Strauss might be a drug-induced illness. In particular, an association has been detected with cysteinyl leukotriene 1 receptor antagonists. However, some authorities appear to regard this seeming association as the result of the "unmasking" of preexisting Churg-Strauss, as the introduction of leukotriene inhibitors permits corticosteroid doses to be reduced (Garcia-Marcos, 2003; Kemp, 2003).

Unlike most noninfectious vasculitides, Churg-Strauss disease is distinctive in its pathology, owing to the abundance of eosinophils in the inflammatory perivenular exudate. The vasculopathy is predominantly an arteriopathy, tending to affect small- and medium-sized arteries much more than arterioles, veins, or capillaries. This predilection also is found in PAN and some other conditions. However, the predominance of eosinophils sets Churg-Strauss disease apart from these other conditions. However, epithelioid and giant cells also are found in the inflammatory exudate of patients with Churg-Strauss disease.

The inflammatory arteriopathy evolves into granulomatous fibrinoid necrosis of the vascular media. The result of this process includes the development of collagenolytic or necrobiotic noninfectious granulomata. The granulomatous material surrounds altered vascular elastic fibers and collagen as well as acellular pigmented debris, which is helpful in pathologically distinguishing one form of granulomatous vasculitis from another. Churg-Strauss disease is associated with "red" collagenolytic granulomas.

Lungs are the chief organs involved in patients with Churg-Strauss vasculopathy, and they almost invariably develop regions of angiopathy as the disease progresses. Typical manifestations include granuloma formation, which occurs within vascular walls and in adjacent pulmonary tissues. Similar angiopathic changes develop in the heart (approximately 85%), skin (70%), peripheral nervous system (66%), central nervous system (60%), kidneys (40%), gastrointestinal tract (40%), and musculoskeletal system (20%).

Vasculitic involvement of the heart is found in approximately 85% of cases, typically manifesting as low-output congestive cardiac failure. Churg-Straus disease should be considered in adults with asthma and eosinophilia who develop chest pain, shortness of breath, and cardiogenic shock (Shanks, 2003).

• Evaluation reveals cardiomegaly, restrictive cardiomyopathy/perimyocarditis, diminished myocardial contractility, and pericardial effusion as factors contributing to heart failure.
• Both systolic and diastolic dysfunction may be discerned.
• Steady decline in myocardial shortening fraction often follows.
• Heart failure is the most important cause of death in Churg-Strauss disease and is a major determinant of prognosis.

Skin and nervous tissues are the systems next most commonly involved in patients with Churg-Strauss disease.

• The skin is involved in 65-70% of cases that have progressed to the systemic vasculitic phase. Small-vessel vasculitis of the skin results in purpura or nodules.
• Eosinophilic granulomatous changes in the wall of the gallbladder may result in obstructive jaundice or pain and require cholecystectomy (Tatsukawa, 2003).
• Approximately 64-80% of patients in the systemic phase of Churg-Strauss disease develop peripheral neuropathy, usually in the pattern of mononeuritis multiplex. Mononeuritis multiplex is the second most common initial manifestation of systemic vasculitis in adults with Churg-Strauss disease.
• Initial mononeuritic findings often progress to asymmetrical polyneuropathy, which is restricted to the limbs. As with PAN, both motor and sensory deficits are detectable, especially in the legs, thus, the sciatic nerves (including peroneal and tibial branches) are involved more frequently than radial, median, or cubital nerves.
• Sensory disturbances may include hypoesthesia or hyperesthesia, allodynia, and pain.
• Polyneuropathy may regress with treatment.
• Vasculitis of muscle develops in slightly more than 20% of patients.

Approximately 60% of adults with Churg-Strauss disease develop CNS vasculitis. This is unlike PAN, in which CNS manifestations are rare. However, CNS vasculitis has not been reported in children with Churg-Strauss disease. It does occur in adolescents, albeit rarely.

• Manifestations include intellectual and motor disturbances. These may be abrupt in onset, and seizures may occur.
• Acute cerebral hemorrhage is among the important causes of sudden death in Churg-Strauss disease. In some but not all instances, hemorrhage occurs in individuals who have hypertension.

Gastrointestinal dysfunction develops in 40-60% of patients. Most commonly, this takes the form of mesenteric vasculitis, similar to that seen in PAN.

• The most common manifestations are bloody diarrhea and abdominal pain.

Arteritis of medium-sized blood vessels of the kidneys develops in 20-50% of cases. However, hypertension is less common in Churg-Strauss disease than in PAN.

• As in PAN, the predominant renal pathology is eosinophilic interstitial pauci-immune segmental glomerulonephritis (Tatsukawa, 2003).
• Crescentic necrotic glomerulopathy may develop in some instances, as is also found in microscopic polyangiitis.
• IgA glomerulonephropathy may develop, as also is the case in microscopic polyangiitis, but rarely. This is a pathological change that is more typical of Schönlein-Henoch purpura.
• In early reports of Churg-Strauss disease, uremia was an occasional cause of death. Current management has considerably reduced the risk of uremia, and in most cases of Churg-Strauss disease, renal involvement is mild. Even if the renal involvement is severe, renal disease usually responds well to corticosteroid treatment.

Testicular pain, with or without epididymitis, may occur in men with Churg-Strauss disease.

Frequency

United States

Churg-Strauss disease is said to account for slightly more than 2% of all vasculitic illnesses. The various primary systemic vasculitides (Churg-Strauss disease, PAN, microscopic polyangiitis, Wegener granulomatosis) together are estimated by Watts et al to affect 15-25 per million individuals in North America annually.

International

Little information is available concerning international variation in the prevalence or incidence of Churg-Strauss disease.

Mortality/Morbidity

• The 5-year survival rate reported in the large series of Chumbley and associates is 62%.
• Morbidity is chiefly cardiopulmonary. Painful arthritis, arthralgia, and myalgia are common troublesome and recurrent manifestations. Abdominal pain, diarrhea, gastrointestinal bleeding, and bowel perforation are less common but important complications.Necrotizing glomerulonephritis occurs in fewer than half of the cases. It tends to affect older patients, who as a group have particularly poor outcomes. Early diagnosis of this renal complication improves outcome (Booth, 2003).Leukopenia due to immunosuppressive therapy enhances risk for sepsis and death, hence should be avoided (Booth, 2003).The chief neurological morbidity is, as in PAN, peripheral neuropathy.
• The chief causes of mortality related to Churg-Strauss disease are severe asthma, cardiopulmonary failure, or gastrointestinal complications. In general, the long-term outcome of Churg-Strauss disease does not differ greatly from that of PAN.

Race

Few data are available regarding racial variations in occurrence or severity of Churg-Strauss disease. In some studies, no racial predilection has been suggested; in others, without clear documentation, it has been suggested that Churg-Strauss disease shares with other systemic vasculitides the tendency toward greater prevalence in whites. Clearly additional data are necessary, using data of greater refinement entailing the casting of a wide socioeconomic and geographic net and greater precision in characterizing populations at risk than what may be based upon genetic markers of skin pigmentation.

Sex

• As with a number of other primary systemic vasculitides, males are slightly more likely than females to develop Churg-Strauss disease.
• The male-to-female ratio for Churg-Strauss disease is estimated by some to be approximately 1.4:1, similar to the sex-related risk ratio for the combined class of Churg-Strauss disease, PAN, microscopic polyangiitis, and Wegener granulomatosis. A higher risk ratio for males is found for one of the important differential considerations, hypereosinophilic syndrome (HES). HES is likely a heterogeneous collection of very severe diseases that are very rare and tend to occur in persons aged 20-50 years, with a male-to-female ratio of 9:1. It is not linked with asthma, but pulmonary disease may be found particularly on the basis of cardiac disease or pulmonary embolization.

Age

Most individuals with Churg-Strauss disease experience the onset of disease in the age range of 15-69 years. Peak risk for vasculitic manifestations is in the middle of the fourth decade of life. Thus, the vasculitic stages of Churg-Strauss disease tend to develop at earlier ages than other primary systemic vasculitides, for which the average age of occurrence is about 60-65 years.

• Churg-Strauss disease is chiefly a disease of adults, occurring in individuals with adult-onset asthma. The vasculitic stages of Churg-Strauss disease seldom, if ever, manifest themselves in small children, although they may do so in teenagers. On the other hand, the premonitory asthmatic stage of Churg-Strauss disease may manifest in children as young as 3 years.
• Although occurrence of the subsequent vascular stages of illness has been regarded as rare even in adolescents, some data suggest that as many as 20% of patients may manifest vasculitic findings in the second decade of life, often preceded by bronchial asthma during the first decade.
• Childhood-onset Churg-Strauss disease is less likely to be complicated by central nervous system vasculitis than cases that develop in middle-aged individuals.

Clinical

History

• First phase
  • The first phase is prodromal to the 2 subsequent vasculitic phases. Bronchial asthma is the nearly constant finding of the first phase. The presence of an asthmatic condition (usually steroid-dependent) is the historical feature that most commonly prompts consideration of Churg-Strauss disease in individuals who manifest any of the many vasculitic abnormalities of various organ systems that may be present in ensuing stages of the disease.
  • However, it must be noted that it is possible for asthmatic individuals to develop vasculitic or inflammatory diseases other than Churg-Strauss disease as explanations for the disease of either pulmonary or nonpulmonary organ systems. The occurrence of tissue and blood eosinophilia provides an additional important clue in the individual who has asthma and abnormalities of sudden onset in several organ systems that Churg-Strauss disease is the likely cause of the various organ system disease manifestations noted below.
  • An additional historical fact of importance is the recent addition of leukotriene receptor antagonists to the treatment regimen for asthma. This association is found in perhaps half of the cases, in three quarters of these patients, the addition of those drugs was made within 3 months prior (Kawakami, 2005).
  • It must further be noted that exceedingly rarely, Churg-Strauss pathogenesis may result in disease of various organ systems in individuals who do not have either asthma or eosinophilia (Sharma, 2004).
  • Additional common findings are allergic rhinitis, nasal polyposis, sinusitis, and recurrent bronchitis or pneumonia. Nasal rhinitis and polyposis often precede the onset of reactive airway disease. The severity of asthma ranges from mild to quite severe and steroid-dependent.
  • The duration of the first phase, prior to progression of illness, averages about 28 months (range 4-72 mo). However, a few patients remain in the prodromal stage of illness for 30 years or more before the second or third phase of Churg-Strauss disease manifests.
  • Recurrent fevers of unclear etiology and weight loss, either of which may occur during the first phase of Churg-Strauss disease, enable the careful clinician to suspect that Churg-Strauss disease underlies the allergic and asthmatic manifestations. Another clue is the fact that the reactive airway component of Churg-Strauss disease tends to develop at a later age (commonly, the fourth decade of life) than is typical for idiopathic asthma. However, fairly typical early childhood-onset asthma may presage the development of the vasculitic stages of Churg-Strauss disease in adolescence.
  • The frequency of Churg-Strauss disease–related reactive airway disease increases, and the severity of the disease worsens, as the vasculitic stage of Churg-Strauss disease is reached. In some cases, an unexpected remission of asthma occurs with the onset of vasculitic manifestations of the second phase of Churg-Strauss disease.
• Second phase
  • During the second phase of illness, hypereosinophilia of blood develops in association with tissue eosinophilia and Loeffler syndrome. During this phase, the eosinophils comprise on average 40% of the WBC count on peripheral blood film (range, 18-65%). Particularly characteristic complications of this phase of illness are chronic eosinophilic pneumonia and eosinophilic gastroenteritis. Hemoptysis may occur. Patients experience a relapsing and remitting course of eosinophilic infiltrative disease and blood eosinophilia.
  • In some instances, the second phase of illness consists of the combination of chest pain, shortness of breath, and development of cardiogenic shock. ECG may suggest myocardial infarction, although coronary arteries may appear normal on angiography. Valvular insufficiency and segmental or global hypokinesis of the myocardium may be found. These findings suggest Churg-Strauss myocarditis (Shanks, 2003).
  • Fever always is present during periods of exacerbation.
• Third phase
  • Months to many years of such intermittent bouts precede the third phase, that of systemic vasculitis. Patients with continued reactive airway manifestations may experience remission, often to a remarkable extent, at the onset of this third phase of Churg-Strauss disease. As vasculitis develops and worsens, weight loss may be noted.
  • On average, progression from the initial stage of Churg-Strauss disease to fully developed systemic vasculitis takes about 3 years. In some quite fulminant cases, systemic vasculitic manifestations may develop without a prior second phase of relapsing-remitting complications. In other cases, the second and third phases of Churg-Strauss disease develop simultaneously. The interval between first and third phases of Churg-Strauss disease is prognostically significant. As might be expected, the shorter the duration of that interval, the worse the prognosis.
• Signs and symptoms may include any of the following:
  • Symptoms related to congestive heart failure ranging from congestive heart failure to cardiogenic shock. ECG may suggest myocardial infarction, although coronary arteries may appear normal on angiography. Valvular insufficiency and segmental or global hypokinesis of the myocardium may be found, all in keeping with Churg-Strauss myocarditis.
  • Neurological findings are most commonly peripheral polyneuropathy, usually mononeuritis multiplex pattern with motor and sensory deficits unexplainable by a single central lesion. Deficits tend to be asymmetrically distributed, involving individual peripheral nerves without reference to specific fiber types. The distribution is not in the "glove-stocking" distribution suggestive of toxic neuropathies. Peripheral muscle stretch reflexes may be diminished or lost. Sensory disturbances may include hypoesthesia, hyperesthesia, allodynia, or other forms of pain. Pain and paraesthesia tend to involve the legs. However, other manifestations may result in vasculitic disease of the brain with stroke or hemorrhage. These may result in abrupt onset of motor, intellectual, or sensory deficits, brainstem signs, stupor, or coma. Seizures may occur.
  • Eosinophilic gastroenteritis may result in abdominal pain, weight loss, or bloody diarrhea.
  • Skin changes may include the development of petechiae and palpable purpura.
  • Acute renal failure, developing rapidly over several weeks, is a characteristic feature of the rapidly progressive glomerulonephritis of Churg-Strauss disease. Gross hematuria or pyuria may be found. These findings, due to inflammatory glomerulonephritis, may be misinterpreted as urinary tract infection.
  • Abdominal pain, bloody diarrhea
  • Testicular pain

Physical

• In the first phase of Churg-Strauss disease, examination reveals nasal polyposis, intermittent fever, and findings consistent with sinusitis, allergic rhinitis, and bronchial asthma. The signs of reactive airway disease gradually worsen over time.
• During the systemic vasculitic phase, examination of skin, heart, abdomen, joints, peripheral nerves, and muscles may reveal evidence for the various characteristic changes of Churg-Strauss disease that have been noted in History. Two thirds of patients in this advanced state of illness are found to have cutaneous purpura or nodules.
• Cardiopulmonary findings that are consistent with congestive heart failure, low output state, or pericardial effusion may be discerned.
• Abdominal tenderness and evidence for gastrointestinal bleeding may be found on examination. Occasionally, the examination will reveal evidence of bowel obstruction. The findings of acute abdomen develop in patients with bowel perforation.
• Arthritis may be noted; any joint may be involved.
• Neurologic examination
  • In patients in the systemic vasculitic phase of illness, neurologic examination may reveal evidence of peripheral neuropathy limited to the extremities. At first, the pattern usually is that of mononeuritis multiplex, but with progression of illness, asymmetrical sensory and motor polyneuropathy is found (legs > arms).
  • Neurological findings tend to develop late. Pulmonary manifestations with eosinophilia usually establish the diagnosis of Churg-Strauss disease prior to the development of neurological disease.
  • The examination may disclose CNS manifestations due to intraparenchymal or subarachnoid brain hemorrhages.
  • Optic neuritis, cranial neuritis, and psychosis also have been described in adults with Churg-Strauss disease.
  • Children seldom manifest CNS findings. One girl with Churg-Strauss disease, who was in the early years of the second decade of her life, developed chorea.

Causes

• Genetic factors
  • Several lines of evidence suggest genetic predisposition, which may entail inherited tendency to dysregulation of the cellular immune system. The features of this dysregulation are noted in Pathophysiology.
  • The variation in age at onset of illness is not understood, but suggests the possibility that secondary factors, such as environmental influences, may hasten the onset of disease for some individuals.
• Environmental factors
  • Environmental factors may contribute to the development of Churg-Strauss disease. Thus, for example, the inhalation of fungal spores, such as those produced by actinomycetes and Aspergillus species, has been implicated in the pathogenesis of some cases.
  • Exposure to pigeons and the molds associated with their roosts also may provoke the development of Churg-Strauss disease.
  • Smoking of free-base cocaine was documented carefully as a circumstance preceding individual bouts of an illness similar to or identical to Churg-Strauss disease in one individual. The illness in that case was not sustained endogenously, recurring only with additional episodes of cocaine smoking.
  • Thus, some environmental factors appear to provoke transient effects that resemble Churg-Strauss disease but do not represent a chronic and self-perpetuating disease.
• Drugs
  • Carbamazepine, macrolide antibiotics, and cysteinyl leukotriene-receptor antagonists have been implicated as provocative causes of Churg-Strauss disease. Leukotriene-receptor antagonists may be especially important in terms of provoking chronic Churg-Strauss disease that does not resolve with discontinuation of the inciting drug.
  • Leukotriene-receptor antagonists are used in some patients who are undergoing withdrawal of steroid treatment of asthma. This has prompted some clinicians to ascribe the onset of Churg-Strauss disease to steroid withdrawal rather than to direct effects of the leukotriene-receptor antagonist. They propose that Churg-Strauss manifestations were masked or perhaps prevented by the higher steroid doses. However, several patients who were not in the midst of a steroid taper have developed Churg-Strauss disease after administration of leukotriene-receptor antagonists. Some authorities now recommend the use of inhaled steroids rather than leukotriene-receptor antagonists when attempting to taper systemically administered steroid treatment of asthma.
• Churg-Strauss disease has developed in the wake of Basedow disease with autoimmune thyroiditis; whether this is a chance association is not known.

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