eMedicine Specialties > Dermatology > Diseases of the Vessels

Churg-Strauss Syndrome (Allergic Granulomatosis)

Author: Paula Vogel, MD, Private Practice, Dermatology Associates, San Antonio, Texas
Coauthor(s): Daniel Schissel, MD, Chief, Department of Dermatology, University of Heidelberg Medical School, Germany
Contributor Information and Disclosures

Updated: Nov 1, 2007

Introduction

Background

Allergic granulomatosis and angiitis is a systemic disorder characterized by asthma, transient pulmonary infiltrates, hypereosinophilia, and a systemic vasculitis. Churg and Strauss first described it in 1951, when they reviewed autopsy cases that were previously classified as polyarteritis nodosa. These cases were atypical in that they were associated Churg and Strauss syndrome with asthma and extravascular granulomas, as well as a systemic vasculitis. This disease is now known as allergic granulomatosis and angiitis or Churg-Strauss syndrome (CSS).

Pathophysiology

CSS has been divided into 3 distinct phases, which may or may not be sequential. The prodromal phase is characterized by asthma with or without allergic rhinitis. The second phase is marked by a peripheral blood eosinophilia and eosinophilic tissue infiltration that produces a picture similar to that of Loeffler syndrome, chronic eosinophilic pneumonia, or eosinophilic gastroenteritis. The third, or vasculitic, phase may involve any organ. The most frequent site of involvement is the heart, though other organs that may be involved are the lung, CNS, kidney, lymph nodes, muscle, and skin. Skin involvement occurs in more than two thirds of patients.

The diagnosis is challenging because of the phasic nature and because the 3 histologic features that Churg and Strauss initially described (ie, tissue infiltration by eosinophils, necrotizing vasculitis, and extravascular granulomas) may not be present in all biopsy specimens.

In 1990,¹ the American College of Rheumatology (ACR) developed criteria for epidemiologic and therapeutic studies. These criteria are as follows: (1) asthma, (2) eosinophilia greater than 10% on a differential WBC count, (3) mononeuropathy or polyneuropathy, (4) nonfixed pulmonary infiltrates, (5) paranasal sinus abnormalities, and (6) biopsy containing a blood vessel with extravascular granulomas. The finding of 4 of the 6 criteria yields a sensitivity of 85% and a specificity of 99.7%.

Frequency

United States

CSS is a rare disease and may be underreported.

International

CSS is a rare disease. In the general population, the frequency of the disorder is estimated at 2.4-4 per million patients. CSS may be underreported.

Mortality/Morbidity

The natural history of CSS is variable. The syndrome may range from an indolent process to a rapidly fatal vasculitis.

• Treatment with steroids improves patients' survival, with long-term overall remission rates of 81-82%. However, 26-28% of patients in remission have relapses. The overall mortality rate in treated patients who relapse is only 3.1%.
• A short interval from the onset of asthma to the development of the systemic vasculitis indicates an unfavorable prognosis.

Sex

A slight male predominance exists. The male-to-female ratio is 1.3:1.

Age

The age of onset varies in range of 4-75 with a mean of about 50 years.

Clinical

History

The 3 phases—allergic, eosinophilic, and vasculitic—do not necessarily follow one another in any particular order. Symptoms depend on the phase and organ systems involved.

• Allergic phase
  • Rhinitis, sinus pain, headache
  • Cough
  • Wheezing
• Eosinophilic phase
  • General - Weight loss, fever, sweats
  • Gastrointestinal - Abdominal pain, diarrhea
  • Pulmonary - Cough
• Vasculitic phase
  • General - Malaise, lassitude, fever
  • Cardiac - Chest pain, dyspnea
  • Pulmonary - Cough, hemoptysis
  • Rheumatologic - Arthralgia, arthritis, myalgia
  • Neurologic - Weakness, numbness

Physical

The signs depend on the phase and organ systems involved.

• Allergic phase
  • Nasal polyps
  • Wheezing
  • Cough
  • Rhinitis
  • Sinus tenderness
• Eosinophilic phase
  • General - Weight loss, fever, sweats
  • Pulmonary - Cough, hemoptysis, rales, rhonchi
  • Gastrointestinal - Rebound, masses, obstruction, ascites, bleeding
• Vasculitic phase
  • General - Fever, weight loss, adenopathy
  • Cardiac - Gallop, pericardial friction rub, jugular venous distension, peripheral edema
  • Pulmonary - Rales, rhonchi
  • Nervous system - Mononeuritis multiplex, diffuse peripheral neuropathy (most often in a glove-and-stocking distribution), loss of the visual field, cerebral hemorrhage, infarction
  • Renal - Mild proteinuria and hematuria
  • Genitourinary - Obstructive uropathy
  • Ocular - Episcleritis, panuveitis, marginal corneal ulceration, conjunctival infiltration, retinal infarction
  • Musculoskeletal - Joint swelling, muscle tenderness
  • Cutaneous - Erythematous macules and papules resembling erythema multiforme, hemorrhagic lesions ranging from petechiae to extensive ecchymoses, cutaneous and subcutaneous nodules, facial edema, livedo reticularis, urticaria, vesicles (Different morphologies may occur simultaneously. The lesions tend to occur in crops and may show remissions and exacerbations. Nodules frequently occur on the scalp and extensor surfaces of the extremities.)

Causes

The etiology of CSS remains unclear; however, the presence of asthma, eosinophilia, and increased immunoglobulin E (IgE) levels suggest an allergic process. Some authors have implicated drug sensitivities to penicillin, penicillamine, iodides, leukotriene modifiers, and mesalazine.²

A report from a workshop sponsored by National Institutes of Health concluded that the vasculitis in CSS is unmasked by the tapering of steroids rather than by an adverse effect of the leukotriene modifiers. The development of CSS after hyposensitization vaccination and associated with pulmonary infection suggest that these events could initiate an inflammatory cascade.

• Search for CME/CE on This Topic »