Churg-Strauss Syndrome

Definition
• Churg-Strauss syndrome, also referred to as
allergic angiitis and granulomatosis, was
described in 1951 by Churg and Strauss and is
characterized by asthma, peripheral and tissue
eosinophilia, extravascular granuloma
formation, and vasculitis of multiple organ
systems.
 Incidence and Prevalence

• Churg-Strauss syndrome is an uncommon

disease with an estimated annual incidence of
1–3 per million. The disease can occur at any
age with the possible exception of infants. The
mean age of onset is 48 years, with a female-
to-male ratio of 1.2:1.
 Pathology and Pathogenesis

• The necrotizing vasculitis of Churg-Strauss syndrome

involves small and medium-sized muscular
arteries, capillaries, veins, and venules.
• A characteristic histo-pathologic feature of Churg-Strauss
syndrome is granulomatous reactions.
• These are usually associated with infiltration of the
tissues with eosinophils.
• strong association with asthma and its clinico-pathologic
manifestations, including eosinophilia, granuloma, and
vasculitis, point to aberrant immunologic phenomena.
Eosinophils Infiltration
 Clinical Manifestations
• Often exhibit nonspecific manifestations:
Fever,
Malaise,
Anorexia,
and weight loss.
• The pulmonary finding similar to the asthmatic attacks with
pulmonary infiltration.
• Mononeuritis multiplex is the second most common
manifestation.
• Allergic rhinitis and sinusitis develop in up to 61% of patients
and present on the early course.
• Heart disease occurs in 14% of patients and is an important
cause of mortality.(CHF or Heart attack)
• Skin lesions occur in 51% of patients and
include purpura in addition to cutaneous and
subcutaneous nodules.

• The renal disease in Churg-Strauss syndrome

is less common and generally less severe.
 Transient pulmonary infiltrates in a
patient with Churg-Strauss syndrome
The skin rashes of Churg-Strauss
syndrome
 Laboratory Manifestations
• The characteristic laboratory finding in
virtually all patients is striking eosinophilia.
• Evidence of inflammation as evidenced by:
elevated ESR, fibrinogen, or 2-globulins
• Approximately 48% of patients with Churg-
Strauss syndrome have circulating ANCA that
is usually antimyeloperoxidase.
 ANCA Negative CSS
• Clinical patterns:
– Pericarditis
– Livedo
– Symetrical polyneuropathy
– pleuritis
• Eosinophil rich tissue infiltrates
• IL-10 genetic polymorphisms
 Clinical Criteria for CSS
• Lanham’s criteria (all of the following)
– Asthma
– Peak eosinophilia >1.5 x 109 cells/L
– Systemic vasculitis, two or > extrapulmonary sites
• American College of Rheumatology (4 of the following
in the setting of vasculitis)
– Asthma
– Peak eosinophilia >10% total WBC
– Peripheral neuropathy attributed to vasculitis
– Transient pulmonary infiltrates
– Paranasal sinus disease
– Biopsy showing blood vessels with extravasular eosinophils
 Clinical Criteria for CSS
(continued)
• Chapel Hill Consensus Conference
– Asthma
– Peripheral Eosinophilia
– Eosinophil-rich granulomatous inflammation
involving the respiratory tract
– Necrotizing vasculitis affecting small to medium
vessels
 Treatment
• Glucocorticoids (oral prednisone 40-60 mg/day)
alone appear to be effective in many patients.
• In glucocorticoid failure or in patients who
present with fulminant multisystem disease, the
treatment of choice is a combined regimen of
daily cyclophosphamide and prednisone.
• High doses of intravenous steroids (usually
methylprednisolone) maybe useful for those who
failed in the above 2 lines.
 Factors associated with poor
prognosis in CSS
• Factor 5 score (French Vasculitis Study Group)
– Elevated Serum Creatinine (> 1.58mg/dl)
– Proteinuria
– Severe GI tract involvement
– Cardiomyopathy
– Central Nervous System involvement

No factors present = five year mortality 12%

1 factor= five year mortality of 25%
> 2 factors= five year mortality of 46%
 Facts or Fiction?
Controversies in Churg-Strauss
Syndrome
1. Perinuclear (p) ANCA has limited utility in the
diagnosis of CSS
2. pANCA levels do not correlate with disease
activity
3. CSS carries a grave prognosis.
4. Leukotriene receptor antagonists exacerbate
CSS, playing a role in pathogenesis.
 Key Considerations
• Histologic evidence is needed in the diagnosis and as
prognostic guides for CSS
• Surrogate markers of vasculitis can be applied in the
proper clinical setting
• While only positive in 40%, the ANCA has prognostic
value in CSS
• Without poor prognosis factors, treatment need not
be aggressive