Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss Syndrome) Clinical Presentation

Updated: Dec 24, 2018
  • Author: Spencer T Lowe, MD; Chief Editor: Herbert S Diamond, MD  more...
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Presentation

History

Eosinophilic granulomatosis with polyangiitis (EGPA) has three phases, as follows:

  1. Allergic rhinitis and asthma
  2. Eosinophilic infiltrative disease (eg, eosinophilic pneumonia or gastroenteritis)
  3. Systemic medium- and small-vessel vasculitis with granulomatous inflammation

The vasculitic phase usually develops within 3 years after the onset of asthma, although it may be delayed for several decades. The most prominent symptoms and signs are those related to pulmonary, cardiac, dermatologic, renal, and peripheral nerve involvement. Mononeuritis multiplex is a major clinical finding.

The following symptoms and signs of the disease were reported by Guillevin et al in their case series [3] :

  • Constitutional symptoms - Malaise, fatigue, flulike symptoms, weight loss (70%), fever (57%), myalgias (52%)

  • Asthma symptoms - Asthma is a central feature of EGPA, occurring in 97% of patients. Asthma may precede vasculitis by up to 10 years or, less frequently, may coincide with the appearance of vasculitis. Asthma symptoms are usually persistent; therefore, patients are usually treated with steroids. This, in turn, might mask other features of the syndrome. [21]

  • Paranasal sinusitis (61%) - Usually responds to oral steroids

  • Allergic rhinitis - This is a common symptom. Additionally, recurrent sinusitis and polyposis are seen, but unlike in granulomatosis with polyangiitis [Wegener granulomatosis], necrotizing lesions of the upper airway are unusual.

  • Pulmonary symptoms (37%), including cough and hemoptysis

  • Arthralgias (40%)

  • Skin manifestations (49%)

  • Purpura - Skin nodules, urticarial rash, necrotic bulla, digital ischemia

  • Cardiac manifestations - Symptoms related to heart failure, myocarditis, pericarditis, constrictive pericarditis, and myocardial infarction

  • Gastrointestinal (GI) symptoms (31%) - Symptoms related to GI vasculitis, eosinophilic gastritis, or colitis; these include abdominal pain (59%), diarrhea (33%), and GI bleeding (18%).

  • Peripheral neuropathy - Mononeuritis multiplex (most frequent form, occurring in as many as 77% of patients)

  • Less frequent symptoms - Symptoms related to stroke, ophthalmologic involvement, and other rare symptoms

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Causes

Causes of EGPA are unknown. [10]  EGPA is possibly an allergic or autoimmune reaction to an environmental agent or drug.

Several case reports have described drug-induced forms of EGPA. Mesalazine-induced EGPA has been reported in a patient with Crohn disease and sclerosing cholangitis. [22] Publications have addressed the association between propylthiouracil, methimazole, and vasculitides, including EGPA. One report is available on the association of freebase cocaine and EGPA. [23]

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Physical Examination

Except for fever, the physical findings in EGPA are specific to organ-system involvement. Pulmonary involvement is the most prominent. In fact, a pneumonitis plus eosinophilia warrants consideration of this syndrome and a search for evidence of systemic vasculitis elsewhere. [3] In addition to asthma and eosinophilia, a dermato-pulmonary-renal syndrome is the feature of this disease. Mononeuritis multiplex is common.

Skin involvement (60%) may include the following:

  • Leukocytoclastic angiitis with palpable purpura

  • Livedo reticularis, skin necrosis and gangrene, digital ischemia, urticaria, and subcutaneous nodules (See the image below.)

    The skin rashes of Churg-Strauss syndrome. The bio The skin rashes of Churg-Strauss syndrome. The biopsy of this rash showed eosinophilic leukocytoclastic angiitis with poorly formed granulomas.

Upper respiratory involvement may include the following:

  • Allergic rhinitis

  • Paranasal sinusitis

  • Nasal polyposis

Lower respiratory system physical findings are related to the following:

  • Asthma (ie, wheeze), expiratory rhonchi

  • Pneumonitis

  • Hemoptysis secondary to pulmonary alveolar hemorrhage (alveolar capillaritis)

Cardiovascular findings may include the following:

  • Myocarditis and signs related to heart failure

  • Myocardial infarction secondary to coronary vasculitis

Renal findings may include the following:

  • Hypertension

  • Signs of uremia and advanced renal failure

Gastrointestinal findings may include the following:

  • GI bleeding

  • Bowel ischemia and perforation

  • Gastroenteritis

  • Appendicitis

  • Pancreatitis

Nervous system findings may include the following:

  • Peripheral neuropathy (includes mononeuritis multiplex [77%])

  • Central nervous system (includes stroke [5%])

In a study of 15 ANCA-positive and 35 ANCA-negative patients with EGPA, ANCA-positive patients had a significantly higher incidence of renal involvement, skin involvement, and peripheral neuropathy in the form of mononeuritis multiplex at the time of diagnosis. Over the entire follow-up period, ANCA-negative patients had significantly more frequent cardiac manifestations. ANCA-positive patients required significantly higher steroid doses to maintain remission. [24]

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