Named Churg Strauss Syndrome for many years,
this eponym has now been recognized as Eosinophilic granulomatosis with polyangitis (EGPA) by the 2012 Revised International Chapel Hill
Consensus Conference nomenclature of vasculitides.
EGPA, is a rare, non-transmissible, medium-and small-vessel
autoimmune vasculitis that involves mainly the blood vessels of the lungs,
gastrointestinal system, and peripheral nervous system, but also affects the
heart, skin and kidneys.
The worldwide incidence of EGPA is estimated to be 2.5 cases
per 100,000 adults per year. The exact
incidence is unknown in Malaysia. Most patients are between the ages of 40-60 years.
Three phases have been described in the natural history of this disease
– prodromal, eosinophilic, and vasculitic phases, however they do not always
A 45 year-old Malay lady, with
late onset poorly controlled bronchial asthma for 11 years requiring recurrent
hospitalization and allergic rhinitis under otolaryngology follow up first
presented to the orthopedics department with left lower limb numbness and
weakness for 1 week duration.
Clinical examination revealed loss of power for left knee extension and
flexion, dorsi and plantar flexion. Sensation was reduced over left L4, L5, S1
impression of acute prolapsed intervertebral disc with L5/S1 compression and
radiculopathy was made. MRI lumbosacral done revealed lumbar spondylosis,
however there was no spinal canal stenosis and both neural foramina and exiting
nerve roots were preserved. Patient was
discharged home with outpatient physiotherapy.
week later she was readmitted to the medical ward with acute gastrointestinal
symptoms. Incidentally, it was noted that she had multiple vasculitic skin
lesions over trunk, upper and lower limbs. (Figure 1)
1: A group of erythematous papulo-purpuric lesions over both elbows and ankle
investigations revealed leukocytosis (WBC 13.7) with predominant eosinophilia
(35%), elevated erythrocyte sedimentation rate (ESR) 76mm/hr and positive c-ANCA. In view of
these findings a provisional diagnosis of EGPA was made.
biopsy was done and showed leucocytoclastic (hypersensitivity) vasculitis.
Skin epidermis with underlying edematous dermis and
scattered blood vessels showing evidence of vasculitis. (H&E stain,
Dermal edema with neutrophilic infiltrate with karorrhectic
debris within the vessels wall. (H&E stain, magnification x100)
Neutrophilic infiltrate with karyorrhectic debris
within the vessels wall (H&E stain, magnification x200)
Endothelial swelling with fibrinoid necrosis of the
vessels wall with neutrophilic karyorrhectic debris. (H&E stain,
possibility of left lower limb mixed sensory motor deficits occurring due to
mononeuritis multiplex was considered which was subsequently confirmed on nerve
was pulsed with IV Methylprednisolone 500mg OD for 3 days, and then
subsequently put on 1mg/kg prednisolone. Her vasculitic rashes subsided within
was subsequently given 6 cycles of IV Cyclophosphamide 15mg/kg which she
responded very well to and gradually regain full neurological function. Oral
Azathioprine 50mg daily was added later as steroid sparing agent. Her condition
remain stable with normalization of eosinophils count and inflammatory markers.
This case highlights the importance of
considering EGPA among adult patients presenting with neurologic complaint
(left foot drop) and various dermatologic lesions.
In 1990, the American College of Rheumatology (ACR)
proposed the following 6 criteria for the diagnosis of EGPA:
Asthma (wheezing, expiratory rhonchi)
Eosinophilia > 10% in peripheral blood
Pulmonary infiltrates (may be transient)
Histological proof of vasculitis with extravascular eosinophils
Mononeuritis multiplex or
The presence of 4 or more criteria
yields a sensitivity of 85% and a specificity of 99.7%. This patient fulfil 4
out of the 6 criteria.
The asthma seen in EGPA is notable for
being of late onset and frequently severe with no personal or family history of
atopy. p-ANCA provides considerable value in supporting the diagnosis of
clinically diagnosed EGPA. However ANCA positivity is seen only in about 38% of
There is no concensus regarding how EGPA
should be managed. The patient’s prognostic profile determines the choice of
French Five factor score (FFS), revised
2009 is use to stratify patients
Age > 65
Renal insufficiency > 150 umol/l
Absence of ear, nose throat manifestations
Presence of each factor
5 year survival
Corticosteroids alone as induction and
Combination of corticosteroids and
2 or more factor
Treatment for EGPA includes glucocorticoids and other
immunosuppressive agents. In many cases the disease can be put into a clinical
remission. The most reasonable strategy is to
individualize therapy, patient’s profile, clinical presentation.