Churg-Strauss syndrome is a rare eosinophilic vasculitis that was first described in 1951. It is characterized by asthma, eosinophilia, and small to medium vessel vasculitis. Diagnostic criteria require asthma, eosinophilia greater than 10%, and evidence of vasculitis in multiple organs. Treatment involves corticosteroids as the mainstay, with immunosuppressants used for resistant cases. Prognosis is generally good, with a 6.5 year survival rate of 72% and clinical remission in 91% of patients.

1. CHURG STRAUSS SYNDROME
DR. DEVAWRAT BUCHE, MD
FELLOW NATIONAL BOARD ( CCM )
BLK SUPERSPECIALITY HOSPITAL

2. History
• During first half of the century many authors
reported a variant of small vessel vasculitis
similar to PAN.
• Churg and Strauss ( 1956 ),reviewed a number
of autopsy cases earlier coined as PAN.
• They published 3 pathologic features
associated with asthma : eosinophilic
infiltration, granuloma formation and medium
sized vesssel necrotizing vasculitis.

3. Diagnostic Criteria
1. Churg and Strauss :
• asthma
• eosinophilic infiltration
• Granuloma formation
• Small and medium sized vessel vasculitis
2. Lanham’s criteria :
• Asthma
• Peak periphral blood eosinophilia ( > 1.5 × 106)
• Systemic vasculitis involving 2 or more extrapulmonary
organs.

4. 3. American rheumatology association criteria:
• Asthma
• Eosinophilia > 10 %
• Neuropathy
• Non fixed pulmonary infiltrates
• Paranasal sinus abnormality
• Extravascular eosinophils
Criteria requires at least 4 out of 6 criteria.
Sensitivity 85 %, specificity 99.7 %
4. Chapel Hill consensus conference ( 1994 ) definition :
“eosinophil rich granulomatous inflammation involving
respiratory tract and necrotizing vasculitis affecting small and
medium sized vessels and associated with asthma and
eosinophilia”.

5. Epidemiology
• Global distribution
• No gender predilection
• Affects all age groups, predilection for 3rd and
5th decades.

6. Etiology
• Unknown
• Associations with :
1. Inhaled allergens, seasonal predilections
2. Macrolide antibiotics
3. Leukotreine antagonist: montelukast,
zafirlukast
4. Autoimmune mechanism : ? ANCA

7. Clinical Phases
J Am Soc Nephrol 10: 2048–2055, 1999

8. Systemic affections
1.CNS/ PNS
• Occurs in about 78 % patients.
• Mononeuritis multiplex, m.c. of peroneal nerve, ulnar,
radial, internal popliteal nerves.
• Cerebral hemorrhage, infarction
• Despite treatment , neurologic sequlae donot recover fully.
2. RS
• Most common presentation is asthma; it is severe and
requires steroid therapy.
• About 2 % develop airway symptoms after the onset of
vasculitis.
• Cough, dyspnea, sinusitis, allergic rhinitis , alveolar
hemorrhage, hemoptysis.

9. 3. CVS
• Most often the primary target organ, portends a
worse prognosis.
• Cardiomyopathy and heart failure seen in 50%.
• Acute pericarditis, constrictive pericarditis, MI,
other ECG changes may be present.
4.GIT
• Eosinophilic gastroenteritis : abdominal pain,
bleeding, colitis
• Ischemic bowel disease
• Pancreatitis
• cholecystitis

10. 5. Renal
• Proteinuria
• Glomerulonephritis
• Interstitial nephritis
• Renal bx may show FSGN, cresenctic GN or necrosis
• Systemic hypertension
6. Skin
• 50 % patients have skin manifestations
• Palpable purpura, livedo reticularis
• Skin nodules
• Urticarial rashes
7. Other manifestations
• weight loss > 10 % b.w.
• Fever > 38 degree celcius , for > 2 weeks
• Diffuse myalgias
• Migratory polyarthalgia

11. Pathophysiology
• The salient feature : eosinophil associated small vessel vasulopathy.
• The degree of eosinophilic infiltration is in excess of that seen in
ordinary inflammatory states.
• Proposed mechanisms:
1. Activated eosinophils release certain cytokines ( e.g. eotaxin ).
This leads to upregulation of adhesion molecules ICAM 1, VCAM 1.
There is increase in eosinophil binding to activated endothelial cells.
2. Eosinophils amy also be directly responsible for the classical
features of CSS, due to release of its stored cationic proteins : may lead
to cardiotoxicity ( by eosinophilic cationic protein ) and peripheral
neuropathy ( by eosinophil derived neurotoxin )

12. Micrograph showing a vasculitis (Churg-Strauss
syndrome). H&E stain.
Rash seen in Churg Strauss syndrome

13. Diagnostics
1. Systemic eosinophilia : hallmark
• > 10 % is a defining feature. Maybe > 75 % at
presentation , which rapidly responds to
corticosteroid therapy.
2. Clinical value of ANCA : questionable

14. • Increase in IL 2, thrombomodulin ,
eosinophilic cationic protein : correlate with
disease activity.
• Other markers: IL 5, TNF α, CD 25, CD 69, CD
30
J Am Soc Nephrol 10: 2048–2055, 1999

15. Radiologic features
1.CXR
• Bilateral, non segmental patchy infiltrates.
• Reticulonodular or nodular disease without cavitations
• Pleural effusions
• Hilar adenopathy
2. CT SCAN
• B/L ground glass opacity
• Subpleural consolidation with surroiunding ground glass
opacity
• Bronchial wall thickening, hyperinflation, septal thickening,
hilar adenopathy
• Pericardial and pleural effusions.
3. CT ANGIO
• s/o vasculitis in coronary and peripheral vessels and CNS.

16. (A) Posteroanterior chest radiograph shows small bilateral lower lobe consolidation,
linear opacities, and small pleural effusions.

18. Differential diagnoses
J Am Soc Nephrol 10: 2048–2055, 1999

19. Treatment
1. Corticosteroids : mainstay of therapy
• Disease responds rapidly.
• Stroids suppress the gene transcriptionof various
cytokines and inhibit the prolongation of eosinophil
survival in extravascular tissues.
2. Immunosuppressive agents :
• Cyclophosphamide ( oral/ iv) : may be used in patients
with steroid resistant cases of CSS.
• Azathioprine, cyclosporine, immunoglobulin infusions :
inconclusive benefits
• Plasmapheresis ( with steroids alone/ steroids with
cyclophospahmide ): no additional benefits
• Future therapies : monoclonal ab against IL 5.

20. Prognosis
• Overall good prognosis
• 6.5 year survival rate of 72%, with clinical
remission in 91% patients ( Guillevin et al ).
• Long term morbidity relates to sequalae of organ
damage during vasculitic phase or complications
of the immunosuppressive therapy.
• Asthma may persist, slowly progressive heart
failure may develop and peripheral neuropathy
may progress.

21. THANK YOU !!