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Churg-Strauss Disease Clinical Presentation

  • Author: Robert Stanley Rust, Jr, MD, MA; Chief Editor: Amy Kao, MD  more...
 
Updated: Oct 13, 2014
 

History

Lanham divided the clinical evolution of Churg-Strauss disease into 3 phases.[4]

First phase

The first phase is prodromal to the 2 subsequent vasculitic phases. Bronchial asthma is the initial disease manifestation in as many as 80-90% of cases of Churg-Strauss disease.[27] The asthma is severe in as many as one third of all patients. An additional 6% will develop asthma within a year of onset of manifestations in other organ systems. In most series, bronchial asthma is the first clinical feature and is the nearly constant finding of the first phase, preceding fever or eosinophilia. Some authorities have estimated that less than 3% of all individuals who develop CSD have no asthma as an element of their CSD.

  • Additional findings that often precede or accompany the first phase of CSD include allergic rhinitis, nasal polyposis, sinusitis, and recurrent bronchitis, or pneumonia. Nasal rhinitis and polyposis, in particular, are likely to precede the onset of reactive airway disease.
  • 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. [33]
  • 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 other organ systems that may be present in ensuing stages of the disease.
  • Recurrent fevers of unclear etiology and weight loss, either of which may occur in the first phase of illness in more than half of individuals developing CSD, 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.
  • Exceedingly rarely, Churg-Strauss pathogenesis may result in development of the systemic vasculitic stages of CSD in individuals who do not have either asthma or eosinophilia.
  • However, also remember 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 as comprising the systemic vasculitic stages of CSD.
  • 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.
  • The frequency of CSD–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

See the list below:

  • 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. [28]
  • 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 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.
    • Neurologic 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.
    • Testicular pain may occur in men with Churg-Strauss disease.
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Physical

See the list below:

  • 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 the skin, heart, abdomen, joints, peripheral nerves, and muscles may reveal evidence for the various characteristic changes of Churg-Strauss disease as 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 reveals 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).
    • Neurologic findings tend to develop late. Pulmonary manifestations with eosinophilia usually establish the diagnosis of Churg-Strauss disease prior to the development of neurologic disease.
    • The examination may disclose CNS manifestations due to intraparenchymal or subarachnoid brain hemorrhages.
    • Optic neuritis, cranial neuritis, and psychosis 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.
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Causes

See the list below:

  • Genetic factors
    • Several lines of evidence suggest a genetic predisposition, which may entail an inherited tendency to dysregulation of the cellular immune system. The features of this dysregulation are discussed in Pathophysiology.
    • The variation in age at onset of illness is not understood, but this variation 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. 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 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.
    • In summary, 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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Contributor Information and Disclosures
Author

Robert Stanley Rust, Jr, MD, MA Thomas E Worrell Jr Professor of Epileptology and Neurology, Co-Director of FE Dreifuss Child Neurology and Epilepsy Clinics, Director, Child Neurology, University of Virginia School of Medicine; Chair-Elect, Child Neurology Section, American Academy of Neurology

Robert Stanley Rust, Jr, MD, MA is a member of the following medical societies: Child Neurology Society, Society for Pediatric Research, American Headache Society, International Child Neurology Association, American Academy of Neurology, American Epilepsy Society, American Neurological Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Kenneth J Mack, MD, PhD Senior Associate Consultant, Department of Child and Adolescent Neurology, Mayo Clinic

Kenneth J Mack, MD, PhD is a member of the following medical societies: American Academy of Neurology, Child Neurology Society, Phi Beta Kappa, Society for Neuroscience

Disclosure: Nothing to disclose.

Chief Editor

Amy Kao, MD Attending Neurologist, Children's National Medical Center

Amy Kao, MD is a member of the following medical societies: American Academy of Neurology, American Epilepsy Society, Child Neurology Society

Disclosure: Have stock from Cellectar Biosciences; have stock from Varian medical systems; have stock from Express Scripts.

Additional Contributors

Robert J Baumann, MD Professor of Neurology and Pediatrics, Department of Neurology, University of Kentucky College of Medicine

Robert J Baumann, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Pediatrics, Child Neurology Society

Disclosure: Nothing to disclose.

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