2. Case report
A 45-year-old male patient presented with complaints of fever and
myalgias for 7 days duration, parasthesias and weakness of both
hands and feet for 4 days, cough, breathlessness and wheeze for 4
days duration.
He had history of sinusitis, in the past underwent sinus surgery
seven years and then two years back. He was a known asthmatic
using inhaler since five years.
He had no history of Diabetis mellitus, Hypertension, coronary
artery disease in the past.
3. His general examination was unremarkable.
Pulse rate was 108/minutes,
respiratory rate 28/minutes and
blood pressure was 120/70 mm Hg.
Respiratory system examination revealed bilateral expiratory Ronchi.
Nervous system examination revealed bilateral asymmetrical distal muscle
weakness with sensory loss.
Other system examination were normal.
4. Investigations revealed hemoglobin 11.7 mg/dl, total Leukocyte count
21,700 cells/mm3, platelet count 3.0 lakhs/mm3, ESR 70 mm
Eosinophil count was 4000cells/mm3.(normal upto 6oo)
Creatinine phosphokinase was 532U/L (normal upto 200 U/L)
Chest radiograph was normal.
P-ANCA was positive.
5. Introduction
Churg-Strauss syndrome is an extremely rare disorder that
affects small-to-medium-sized vessels that
causes inflammation of your blood vessels.
It is also known as Eosinophilic granulomatosis with
polyangiitis
It was first described in 1951 by Churg and Strauss
Usually the patients' age is middle aged
Both men and women are equally affected
6. causes
The etiology is still unknown, but it has been attributed to
hypersensitivity to an inhaled agent. Rarely a parasitic infection or
antigenic drug for desensitization represents a triggering event.
It most often affects your nose, sinuses, lungs, heart, intestines,
and nerves. It can also affect the kidneys, muscles, or joints.
It is characterized by a triad of clinical signs:
asthma,
hypereosinophilia and
necrotizing vasculitis.
7. Stages and symptoms
The Allergic(prodromal) stage is marked by airway inflammation;
almost all patients experience asthma and/or allergic rhinitis.
symptoms such as rhinorrhea and nasal obstruction, and the
formation of nasal polyps that require surgical removal and
Sinusitis may also be present
The second stage is characterized by hypereosinophilia, which
causes tissue damage, most commonly to the lungs and
the digestive tract. weight loss, night sweats, asthma, cough,
abdominal pain, and gastrointestinal bleeding. Fever
and malaise are often present.
The third stage consists of vasculitis, hallmark of
EGPA, inflammation of the blood vessels, and the consequent
reduction of blood flow to various organs and tissues.
8. Complications
Pheripheral nerve damage – leading to numbness, burning and loss
of sensation in the hands and feet.
Scarring of the skin
Heart related problems- infarction ,pericarditis, myocarditis , Heart
attack and heart failure
GI manifestations- ulcerations, perforation and peritonitis
Kidney damage
9. criteria
The American College of Rheumatology 1990 criteria for diagnosis of Churg–Strauss
syndrome lists these criteria:
Asthma
hypereosinophilia, i.e. eosinophil blood count greater than 1,500/microliter
Damage to 1 or more nerve groups
Migratory spots or lesions on chest X-ray
Presence of paranasal sinus abnormalities
Histological evidence of extravascular eosinophils
A patient shall be said to have EGPA if at least four of these six criteria are positive.
10. Diagnosis
Full blood count
Imaging studies (CT, X-ray)
Biopsy of affected tissue
P-ANCA with indirect ELISA
Other test: ECG,EMG and GI endoscopy
11. Management
There is no cure
steroids (such as prednisone and prednisolone)
immunosuppressive drugs (such as azathioprine,
methotrexate and cyclophosphamide)
Lifestyle changes
On December 12, 2017, the FDA approved mepolizumab, the first
drug therapy specifically indicated for the treatment of EGPA.
12. Prognosis
Without treatment, the disease can be fatal
With treatment, the 1-year survival rate is 90% and the 5-
year survival rate is 62%.
