Churg-Strauss syndrome (Allergic Angiitis and Granulomatosis)

120
J Emerg Crit Care Med. Vol. 21, No. 2, 2010
急 重 症 影 像
Churg-Strauss syndrome
(Allergic Angiitis and Granulomatosis)
Yun-Te Chang 1,2 , Shue-Ren Wann 1 , Chih-Hsiang Kao 1 , Neng-Chyan
Huang 1 , Mei-Chen Liao 1 , Wang-Chuan Juang 1 , Hong-Tai Chang 1
A 25-year-old woman suffered from sinusitis,
asthma and chronic cough intermittently for 1
year was admitted due to persistent worsening
cough for 2-3 weeks. Her examination revealed
a weakness in appearance and initial blood test
results showed marked eosinophilia: WBC of
14100/cumm, neutrophil at 49%, lymphocyte at
13%, and eosinophil at 32%. Chest x–ray showed
bilateral pulmonary nodules (Fig. 1) and computed
tomography (CT) scan also demonstrated multiple
peripheral pulmonary nodules, which suggested
chronic eosinophil pneumonia (Fig. 2). However
during hospitalization, her sputum TB, fungus, and
bacteria cultures all showed no growth. In addition,
sputum cytology, serum anti-nuclear antibody,
antineutrophil cytoplasmic autoantibody and serum
rheumatoid factor results were all negative. Bone
marrow biopsy demonstrated eosinophilia and
serum total eosinophil count increased up to 13030
/cumm. After treatment with prednisolone, she was
discharged under the diagnosis of Churg-Strauss
syndrome in a stable condition and her follow-up
CT scan showed almost complete remission.
Fig. 1
Chest x–ray showing bilateral pulmonary
nodules
Received: April 20, 2009 Accepted for publication: July 17, 2009
From the 1 Department of Emergency Medicine, Kaohsiung Veterans General Hospital
2
National Yang-Ming University, School of Medicine
Address reprint requests and correspondence: Dr. Shue-Ren Wann
Department of Emergency Medicine, Kaohsiung Veterans General Hospital
386 Tachung 1st Road, Kaohsiung 813, Taiwan (R.O.C.)
Tel: (07)3468342 Fax: (07)3468343
E-mail: vghks1109@yahoo.com.tw

Churg-Strauss syndrome
121
Fig. 2
Chest CT scan demonstrating multiple peripheral pulmonary nodules
Comments
Churg-Strauss syndrome is a rare systemic
vasculitis and the clinical diagnostic criteria
are asthma, blood eosinophilia greater than
1500/μL, and of vasculitis involving two or
more extrapulmonary organs. In addition,
allergic rhinitis, nasal polyps, and sinusitis are
common accompanying features. The lungs, skin,
and nervous system are the most common sites of
involvement for patients with this disease (1-2) . The
original pathologic description reported by Churg
and Strauss and the classic pathologic findings
in the lung include a combination of eosinophilic
pneumonia, granulomatous inflammation, and
vasculitis (3)
The most common chest radiographic
findings include transient patchy alveolar
opacities, while diffuse interstitial infiltrates or
nodular densities occur infrequently (4) . Most
p a t i e n t s a r e t r e a t e d w i t h c o r t i c o s t e r o i d s,
although immunosuppressive drugs, usually
cyclophosphamide, may be added in some cases.
The prognosis is good, however, remission
occurs in the majority of patients. Cardiac
involvement with myocardial infarction or
congestive heart failure is the most common
cause of death (5) .
References
1. Lanham JG, Elkon KB, Pusey CD, et al. Systemic
vasculitis with asthma and eosinophilia:
a clinical approach to the Churg-Strauss syndrome.
Medicine 1984;63:65-81.
2. Katzenstein AL. Diagnostic features and differential
diagnosis of Churg-Strauss syndrome in
the lung. Am J Clin Pathol 2000;114:767-72.
3. Churg J, Strauss L. Allergic granulomatosis,
allergic angiitis, and periarteritis nodosa. Am J
Pathol 1951;277-94.
4. Choi YH, Im JG, Han BK, et al. Thoracic
manifestation of Churg-Strauss syndrome:
radiologic and clinical findings. Chest
2000;117:117-24.
5. Lhote F, Guillevin L. Polyarteritis nodosa, microscopic
polyangiitis, and Churg-Strauss syndrome:
clinical aspects and treatment. Rheum
Dis Clin North Am 1995;21:911-47.