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Eosinophilic polymyositis.

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Eosinophilic Polymyositis
Robert B. Layzer, MD, Martin A. Shearn, MD, and Saty Satya-Murti, MD
I n 3 patients with subacute polymyositis, muscle biopsies were remarkable for showing abundant infiltration by
eosinophils. I n each case the inflammatory myopathy was part of a systemic illness known as the hypereosinophilic
syndrome (HES). T h e systemic manifestations included eosi nophilia, anemia, hypergammaglobulinemia, vascular
involvement (subungual petechiae, livedo reticularis, Raynaud’s phenomenon), skin rash and subcutaneous edema,
pulmonary infiltrates and pleuritis, cardiac involvement (congestive heart failure, arrhythmias, heart block, pericarditis, myocardial and endocardial fibrosis), and peripheral neuropathy. Myopathy has received scant mention i n
previous reports of HES, but 30% of the patients had neurological symptoms such as encephalopathy, multiple
cerebral infarcts (possibly embolic in origin), peripheral neuropathy, and mononeuropathy multiplex. T h e prognosis
is poor, average survival being only nine months after the onset of symptoms; but 2 of our patients, and a few reported
by others, have responded well to corticosteroid treatment.
Layzer RB, Shearn MA. Satva-Murti S Eosinophilic polymyositis Ann Neurol 1:65-7 1, 1977
‘This patient was previously reported in Medical staff conference:
eosinophilia and eosinophilic cardiris. Calif Med 111:388-395,
1969.
surrounded by eosinophils. Random, segmental degeneration of muscle fibers was occasionally noted. In a smear from
a bone marrow aspiration, eosinophils composed 13% of
nucleated blood cells. The white cell count was 7,500 with
2 1% eosinophils, and the serum creatine phosphokinase
(CPK) activity was 838 units. A n EKG showed left bundlebranch block. A skin test for trichinosis and serological tests
for trichinosis, schistosomiasis, ascariasis, and visceral larva
migrans were all negative.
Because of increasing weakness in the arms and legs, the
patient was admitted to the IJniversity ofCaliforniaHospital
on May 10, 1968. The upper eyelids had a violaceous hue,
and Raynaud’s phenomenon was present in the fingers.
Muscle atrophy was generalized. He was unable to rise from
a supine position, and he had moderately severe weakness
affecting all limb muscles but greater proximally. The muscles were not swollen or tender, and there were no contractures. The muscle stretch reflexes were diminished or absent, and vibratory sensation was reduced i n the toes. The
white blood cell count was 7,500 with 10% eosinophils.
Rheumatoid factor was present in the serum at a dilution of
1 :200.The erythrocyte sedimentation rate was 21 mm per
hour. The serum CPK was 130 and 240 units (normal, less
than 57 units). The EKG was interpreted to show 2 : l
atrioventricular block, incomplete right bundle-branch
block, and an old anterior myocardial infarct. A cine
esophagogram showed a mild, nonspecific esophageal
motility disorder and a large gastric ulcer. Electromyography demonstrated typical myopathic abnormalities together with numerous fibrillation potentials.
Following treatment with a bland diet and antacids, the
gastric ulcer healed completely. In September, 1968, prednisone administration was started in a dosage of 40 mg per
day, but the patient soon noted increased calfpain. Because
of the sudden onset of pain, tenderness, and discoloration
over the right tibia, he was readmitted on September 12. O n
the same day he began to have attacks of Adams-Stokes syn-
From the Departments of Neurology and Medicine, Universiry of
California School of Medicinc, San Francisco, and KaiserPermanerite Medical Center, Oakland, CA.
Address reprint requests ro Dr Layzer, Department of Neurology,
794-M, Gniversity of California, San Francisco, 3rd and Parnassus,
San Francisco, C A 94143.
T h e inflammatory infiltrate of polymyositis typically is
composed of lymphocytes, plasma cells, histiocytes,
and occasional neutrophils. Eosinophilic infiltration
of muscle is rare except in parasitic infections
(trichinosis, cysticercosis, echinococcosis) and in the
arterial lesions of polyarteritis nodosa [ l , 21. In this
report we describe 3 patients with eosinophilic
polymyositis accompanied by eosinophilia of the
blood and involvement of other organ systems. These
patients fit into the syndrome known as disseminated
eosinophilic collagen disease, sometimes called
eosinophilic leukemia or the hypereosinophilic syndrome (HES). In addition to muscle, t h e central and
peripheral nervous systems may be involved in this
condition, which is virtually unknown in t h e neurological literature. A n abstract of this work has been published [2a].
Case R e p o r t s
Putient 1 *
A 46-year-old man complained of progressive muscle pain
and weakness. Two years before admission his calf muscles
had become stiff, swollen, and painful, and the blood
eosinophil count was noted to be 12%. Weakness and pain
spread to the thighs, and Raynaud’s phenomenon appeared
in the hands. In September, 1967, eight months before this
admission, a muscle biopsy was performed at an outside
hospital that revealed marked perivascular and interstitial
inflammatory infiltration composed mostly of eosinophils
and, to a lesser extent, of plasma cells and lymphocytes (Fig
1). Individual frbers and groups of fibers were frequently
Accepted for publication July 7,1976
65
Fig 1 . (Patient 1 .) First muscle biopsy, showing
interstitial infiltration Ly eosinophils and segmental
necrosis. Sectioning artifact is noticeable. (Hematoxylin &
eosin; ~ 1 0 before
0
170% enlargement.)
F i g 2. (Patient 1 .) Autopsy specimen of muscle showing
perivascular infiltration by lymphocytes and plasma cells
without eosinophils. (Hematoxylin G eosin; ~ 1 0 before
0
170% enlargement.)
Fig 3. (Patient 2.)Muscle biopsy showing periuascular
inflammatory infiltrate, predominantly eosinophilic.
(Hematoxylin G eosin; x I00 before 170 % enlmgement.)
F i g 4. (Patient 3.) Resolving splinter hemorrhages are
present under thefingemUil.r.
Fig 5 . (Patient 3.) Muscle biopsy showing nodular
granuloma and interstitial infiltration by eosinophils.
0
170% enlargement.)
(Hematoxylin C eosin: ~ 4 before
F i g 6. (Patient 3.) Muscle biopsy showing a degenerating
muscle fiber invaded by phagocytes, eosinophils,
lymphocytes, and plasma cells. (Hematoxylin 0 eosin;
X 2 5 0 before 170%' en1arEernent.i
cope, and the E K G showed complete heart block. A cardiac
pacemaker was inserted. The sedimentation rate was now 2
mm per hour, the white blood cell count was 9,400 with 2%
eosinophils, and the serum CPK activity ranged from 146 to
356 units. Rheumatoid factor was present in serum diluted
1:400, but the antinuclear antibody test was negative. A
second muscle biopsy in October, 1968, one year after the
first biopsy, showed a marked increase in the extent of
muscle fiber degeneration. In many muscle fibers there were
necrotic segments infiltrated by histiocytes. Many fibers
were rounded, and about 15% of the muscle fibers contained central nuclei. Regenerating fibers were also present
in abundance. Interstitial and perivascular inflammatory
infiltration was seen, but eosinophils were conspicuously
absent. There was an increased amount of fibrous tissue
between the fascicles.
Treatment with prednisone was stopped after four weeks
because of recurrence of the gastric ulcer. Eosinophilia
never returned, but symptoms of ischemia in his fingers
grew worse and a red, scaly rash appeared on his hands and
arms, most prominent over the elbows, with livedo reticularis of the legs. He was readmitted in April, 1969, for
repeated episodes of dyspnea. A lung scan was thought to
show evidence of multiple pulmonary emboli. The cardiac
pacemaker was repositioned. Anticoagulation treatment
was attempted but was abandoned because of erratic control. In July, 1969, he was admitted with severe anterior
chest pains and dyspnea. A pericardial friction rub was
heard, but chest roentgenograms and a lung scan showed no
abnormalities. The patient died suddenly four days after
admission, three years after the onset of symptoms.
At autopsy, the heart weighed 480 gm and was covered
with a fibrinous exudate. The right ventricular wall measured 0.6 cm and the left, 1.3 cm. Areas of yellow white
discoloration were present throughout the walls of both
ventricles. No mural thrombi were detected. Microscopical
sections of the heart revealed scattered foci of old, dense
fibrosis in myocardial tissue on both the left and right sides.
A large area of scarring was present in the left ventricle and
was associated with moderately severe endocardia] fibrosis.
No myocardial inflammation was evident. Sections of
skeletal muscle showed scattered degeneration and
phagocytosis of muscle fibers, and some attempts at regeneration were seen. There was a minimal amount of focal
perivascular and interstitial infiltration with lymphocytes,
but eosinophils were absent (Fig 2). No notable lesions were
found in the other organs.
gradual resolution of the pulmonary infiltration. In 1974, six
months before this admission, right lower lobe pulmonary
infiltrates with pleural effusion appeared. Pleural fluid contained 4,500 white cells/mm3, of which 90% were
eosinophils. The peripheral blood count was 13,900 white
cells/mm3 with 40% eosinophils. Prednisone was administered in a dosage of 30 mg daily for three months; the lungs
cleared and peripheral eosinophilia disappeared.
The patient's recent admission was precipitated by increasing shortness of breath and profound muscle weakness
for one month. Shewas unable to walk without assistance, to
lift her arms to comb her hair, or to rise from a sitting or
supine position. Orthopnea had increased and she experienced paroxysmal nocturnal dyspnea. The pertinent physical findings included multiple splinter hemorrhages under
the fingernails, evidence of cardiac failure with an S, gallop,
and rales at the posterior right lung base. There was striking
weakness of all muscle groups but predominantly those of
the proximal extremities. Pertinent laboratory fiiidings included an initial white blood cell count of 6,700 with 555%
eosinophils. Rheumatoid factor was positive at 1 : 80, and
the antinuclear antibody test was positive with a diffuse
pattern. Hemoglobin was 12 gm/dl on admission, later
falling to 9.5 gm/dl. Fibrin split products were increased in
the urine. The platelet count was 335,000; prothrombin
time, 85'7;; and fibrinogen, 235 mg/dl. Serum enzymes,
including CPK, were within normal limits. A biopsy of the
left quadriceps muscle revealed moderate numbers of muscle fibers undergoing segmental degeneration and invasion
by histiocytes, plasma cells, and eosinophils. There was
prominent perivascular inflammatory infiltration, eosinophils being the dominant cell type (Fig 3). An electromyographic study revealed myopathic potentials and low
voltage of the motor units. Many complex polyphasic potentials were seen. The findings were consistent with
generalized myopath y. Motor nerve conduction velocities
were normal.
Prednisone administered at a dosage of 80 mg per day
resulted in gradual improvement in muscle strength and
disappearance of eosinophilia. The prednisone was tapered
over a period of 10 months LO 10 mg every other day.
Presently she requires no assistance for various activities of
daily living but uses a cane for walking.
Recent laboratory studies reveal normal levels for serum
complement, serum anti-DNA antibodies, quantitative
immunoglobulins, and serum enzymes. The white cell count
is now 5,000 with 2% eosinophils.
Patient 2
A 70-year-old woman was admitted to the hospital with
Patient 3
A 47-year-old woman developed weakness and tenderness
shortness of breath and marked muscle weakness. The
patient had a 40-year history of chronic allergic bronchial
asthma and seasonal hay fever. Five years before, she had
been admitted to the hospital with bilateral pulmonary
infiltrates; at that time the white count was 9,300 with 45%
eosinophils. The hospital course had been complicated by a
variety of cardiac arrhythmias, including multiple premature
ventricular contractions, wandering pacemaker, and coronary sinus rhythm. No basis for the pulmonary lesions was
uncovered, and a diagnosis of Loffler's syndrome was entertained. Prednisone was administered for 15 days with
of the proximal musculature during the month before admission, associated with swelling of the periorbital area,
neck, and hands. Past history included bronchial asthma for
three years, hay fever for four years, and recurrent nongranulomatous anterior uveitis for five years. Laboratory
study showed a white blood cell count of 15,400 with 5 176
eosinophils. The CPK was elevated to 129 units (normal,
less than 80).
The initial hospital examination revealed pitting edema of
the hands, feet, neck, and supraorbital areas. Numerous
splinter hemorrhages were present under the fingernails of
Layzer, Shearn, and Satya-Murti: Eosinophilic Polymyositis
67
both hands (Fig 4 ) . The patient had marked weakness of the
proximal musculature of both upper and lower extremities.
She was unable to raise her head from the horizontal position, and she could not elevate one leg to 45 degrees for
more than five seconds.
Pertinent laboratory findings included hemoglobin, 12
g d d l ; white blood cell count, 12,500 with 55Cz eosinophils; negative antinuclear antibody test; and latex
agglutination titer for rheumatoid factor, 1 : 20. Protein
electrophoresis revealed a polyclonal increase of gamma
globulin to 2.07 gmidl with decrease in serum albumin
to 2.7 gm/dl. Fibrin split products were increased in the
urine. The platelet count was 303,000; prothrombin time,
100%; and fibrinogen, 220 mgidl. Serum complement,
trichinella agglutinins, and an LE preparation were normal.
The erythrocyte sedimentation rate was 3 3 mm per hour
(Westergren). Lactic dehydrogenase was 706 units and
CPK, 145 units. A muscle biopsy performed on the left
forearm revealed a striking picture of granulomatous infiltrates and muscle fiber necrosis. There were several nodular
granulomas .composed of a central amorphous eosinophilic
core surrounded by a ring of phagocytes arranged in
palisades, which i n turn was enclosed by a layer of plasma
cells and eosinophils (Fig 5). Elsewhere there were perivascular collections of plasma cells and eosinophils. Many
eosinophils streamed away from these areas, insinuating
themselves between fascicles and surrounding individual
muscle fibers. Adjacent to the granulomas and inflammatory
infiltrates,focally intense muscle fiber necrosis and regeneration were evident. Scattered single degenerating and regenerating fibers were also present, the former invaded by
phagocytes, eosinophils, and plasma cells (Fig 6).
Prednisone, 40 mg per day, was started on the third
hospital day. The swelling in the hands and face rapidly
abated, muscle tenderness disappeared, and muscle strength
began to improve by the sixth hospital day. No new splinter
hemorrhages appeared. The eosinophil count decreased to
2% and the CPK to 23 units. The patient gradually became
able to perform sit-ups and to rise from a seated position
without help. Prednisone was tapered and after eight
months was withdrawn; however, two months later, fatigue
and muscle weakness returned and blood eosinophils rose
to 16%. Prednisone was reinstituted, followed by prompt
regression of symptoms as well as eosinophilia. The patient
continues to be asymptomatic o n a regimen of 15 mg of
prednisone per day.
Discussion
These patients presented with the usual clinical features of polymyositis, namely, subacute onset of muscle pain and proximal weakness, elevated serum enzymes in 2 cases, and electromyographic findings of
myopathy. Muscle biopsies showed typical changes of
muscle fiber degeneration and regeneration accompanied by profuse infiltration by inflammatory cells.
Instead of the usual mononuclear cells, however, the
inflammatory infiltrates were composed largely of
eosinophils, sometimes perivascular in location and
sometimes interstitial. Furthermore, in each case
there was striking eosinophilia of the blood, ranging
68 Annals of Neurology Vol 1 No 1 January I 9 7 7
from 21 to 5596, with associated signs of syscemic
disease including anemia, hypergammaglobulinemia,
vascular involvement (subungual petechiae, livedo
reticularis, Raynaud’s phenomenon), skin rash and
subcutaneous edema, pulmonary and pleural involvement, cardiac involvement (congestive heart
failure, arrhythmia, heart block, pericarditis, myocardial and endocardial fibrosis), and peripheral
neuropathy. These atypical features-eosinophilia
of
the blood, eosinophilic infiltration in muscle, and
visceral involvement-indicated
that the inflammatory myopathy was part of a generalized disorder different from either ordinary polymyositis or the common collagen vascular diseases.
Visceral involvement is rare in ordinary polymyositis, although inflammatory skin changes occur
in about 40% of the patients [2]. There may be
low-grade fever, and Raynaud’s phenomenon is seen
in up to one-third of the patients [2]. Arthralgia is
common, but objective arthritis is unusual. A few
patients have had pulmonary [3], cardiac [4, 51, or
intestinal involvement [2]. Aside from elevated levels
of serum enzymes, laboratory abnormalities are
sparse; the sedimentation rate is increased in fewer
than half of the patients, leukocytosis may occur,
serum alpha-2 and gamma globulins are frequently
increased, and rheumatoid factor is often present in
serum, but the antinuclear antibody test is usually
negative [2]. When polymyositis is associated with
one of the collagen vascular diseases such as lupus
erythematosus, scleroderma, rheumatoid arthritis,
S jogren’s syndrome, or mixed connective tissue disease, the systemic manifestations and laboratory abnormalities are those of the underlying disease.
Eosinophilia of the blood sometimes occurs in Sjogren’s syndrome and in rheumatoid arthritis [6] but
without eosinophilic infiltration of tissues. Blood
eosinophilia is also a feature of polyarteritis nodosa,
and in the acute phase arteries are infiltrated by neutrophils and eosinophils. Muscle involvement consists
of microinfarction and denervation, however; true
myositis does not occur [2].
Trichinosis could produce many of the findings that
were present in our patients [ 7 ] .In typical cases, gastrointestinal symptoms are followed by fever, urticaria, orbital edema, subungual petechiae, myalgia,
and muscle weakness. Pulmonary, cardiac, and cerebral involvement occur in severe cases. Eosinophilia
of the blood, which may rise to 70’96, persists for
months; depending upon the stage of the illness, muscle biopsy shows various degrees of muscle fiber degeneration and regeneration as well as inflammatory
infiltrates of neutrophils, plasma cells, lymphocytes,
histiocytes, and eosinophils together with increased
interstitial fat and connective tissue. In some cases
trichina larvae are very scarce and eosinophils are not
numerous, so that a mistaken diagnosis of idiopathic
polymyositis may be made [8]. However, trichinosis is
a monophasic illness in which the symptoms reach a
peak between four and six weeks after infection with
gradual improvement afterward. Two of our patients
(Nos. 1 and 2) had a prolonged illness that extended
over several years, and trichinella skin tests and
serological tests were negative in the third patient.
The patients presented here resemble others who
have been reported with a rare multisystem disease
characterized by eosinophilia of the blood and widespread organ involvement variously termed
eosinophilic leukemia, disseminated eosinophilic collagen disease, or the hypereosinophilic syndrome.
The classification of this disorder has undergone considerable evolution since Loffler described the association of eosinophilia of the blood with pneumonitis
[9] and endomyocarditis [ 101, two common features
of HES. Controversy continues as to whether true
eosinophilic leukemia accounts for some cases [ 111,
but most authors agree with the view advanced by
Engfeldt and Zetterstrom [12] that the syndrome is
more closely allied to autoimmune diseases than to
leukemia. In a recent detailed review, Chusid et a1
[13] analyzed 57 published cases and presented 14 of
their own. From their analysis, HES emerges as a
grave disorder largely affecting males (who made up
93% of the cases) and starting at any age between 5
and 80 years with a peak incidence in the fifth decade.
The early symptoms included fever, anorexia, weight
loss, fatigue, arthralgia, abdominal pain, cough,
peripheral edema, skin rash, and neurological abnormalities. Cardiac involvement was detected in most
cases, producing congestive heart failure, valve murmurs, EKG abnormalities, and arrhythmias. Most of
the patients had hepatomegaly and splenomegaly, and
pulmonary involvement occurred in 4096, while skin
rash, lymphadenopathy, and renal impairment were
less common. In most cases the leukocyte count was
increased, and eosinophils constituted more than
30% of the total. Anemia was common, and the
erythrocyte sedimentation rate and serum globulins
were often increased.
At autopsy, cardiac disease was invariably present
and most often consisted of endocardial fibrosis with
adherent thrombi indistinguishable from the lesions
of Loffler’s endocarditis; focal areas of myocardial necrosis and fibrosis were apparently related to small
arterial occlusions, but eosinophilic infiltration of the
heart was present at death in only 13%. Eosinophilic
infiltration was common in liver, spleen, and lungs.
Although thrombosis of small vessels and numerous
small areas of infarction o r necrosis were encountered
in many organs, true vasculitis was rarely seen at autopsy. Some vascular occlusions may have been embolic in origin, but endocardial thrombus was not
present in every case. Disseminated intravascular
coagulation has been proposed as a mechanism, without laboratory documentation [ 141.
Muscle involvement has received scant mention in
previous reports of HES. There are frequent allusions
to muscle pain, tenderness, and weakness, but descriptions of the muscle examination are lacking. In
some patients with symptoms involving muscle,
biopsy [ 131 or autopsy [ 12, 151 revealed intense rnuscle infiltration by eosinophils, but no mention was
made of muscle fiber degeneration or regeneration.
The present report thus constitutes the first detailed
examination of skeletal muscle involvement in HES.
I t appears that overt myopathy may be present more
often than has previously been recognized. In fact,
myopathy may be the presenting feature, or the illness
may pass through a phase in which myopathy is the
dominant problem. In such cases it would be easy to
make a mistaken diagnosis of ordinary polymyositis,
overlooking the more serious implications of a multisystem disorder with eosinophilia.
Other neurological symptoms have been mentioned in 30Yh o f the reported cases [ 131. The most
common syndrome was a diffuse encephalopathy
characterized by confusion, psychosis, seizures, or
coma. Other patients had multiple episodes of sudden
focal cerebral deficit, suggesting vascular insults.
Gardner-Thorpe et a1 [16] reported 4 patients with
Loffler’seosinophilic endocarditis who manifested Balint’s syndrome (optic ataxia and paralysis of visual
fixation) resulting from bilateral parietooccipital lesions. The pathological basis of the cerebral symptoms
consisted of multiple small infarcts associated with
fibrotic occlusion of small arteries without vasculitis.
Peripheral neuropathy and mononeuropathy multiplex have also been reported.
Especially interesting was the occurrence of subungual petechiae (splinter hemorrhages) in 2 of our patients. Nail bed splinter hemorrhages were described
in 2 earlier patients [lG, 171, 1 of whom also had microinfarcts of the finger pads. Other patients have
manifested a generalized petechial rash [16, 18, 191.
Petechiae of this kind are best known in bacterial
endocarditis, but they occur in many other conditions
in which there is occlusion of small blood vessels,
either by emboli or from thrombosis in situ. These
include rheumatic valvular disease without endocarditis [20], trichinosis [ 71 (presumably because of accompanying vasculitis), and collagen vascular diseases
[6]. In HES, either embolism from mural thrombi or
local small vessel occlusive disease could account for
petechial eruptions. Our third patient (No. 1) had
signs of peripheral vascular insufficiency in t h e form
of Raynaud’s phenomenon and livedo reticularis,
suggesting disease of slightly larger arteries.
Raynaud’s phenomenon occurred in 1 of Roberts’s
Layzer, Shearn, and Satya-Murti: Eosinophilic Polymyositis
69
patients [18], and Novack e t a1 [all described 2 patients with HES who manifested extensive calcification of large and medium-sized arteries, producing
peripheral vascular insufficiency with gangrene of digits and intermittent claudication.
Eosinophilia occurs in many disorders not usually
classified with HES, principally parasitic infestations
and allergies; but it is not always easy to define the
boundaries separating these conditions. This difficulty
derives from uncertainty about the pathogenesis of
HES, especially about the role of eosinophils themselves in causing tissue damage. Ive et a1 [22], inves.
tigating African endomyocardial fibrosis, found that
filariasis was present in 36 (915%)of 40 autopsied
cases, and Roberts et a1 [ 181 suggested that the African
disease may be a late stage of Loffler’s endocarditis
brought about by the prolonged eosinophilia of filarial
infection. A history of bronchial asthma (present in 2
of our patients, both women) was mentioned in 16%
of published cases of Loffler’s endomyocarditis reviewed by Frenkel et al [23]; half of the patients with
combined disease were female, although 7896 of all
patients with Liiffler’s endocarditis were male. Loffler’s
endocarditis has also been reported in 2 patients with
tumor-induced eosinophilia, 1 having Hodgkin’s disease [ 141 and the other, acute lymphoblastic leukemia
[ 2 4 ] .A benign syndrome of eosinophilia with inflammatory induration of subcutaneous tissues has been
described; in 1 case, the inflammatory process exrended into the muscle and an electromyogram
showed myopathy, but muscle weakness was not described [251.
The syndrome termed allergic granulornatosis by
Churg and Strauss [ 2 6 ] ,consisting of severe asthma,
fever, hypereosinophilia, and symptoms of damage in
many different organs, is especially difficult to classify.
Chusid et a1 [131 elected not to include the cases of
Churg and Strauss in their review of HES, but Engfeldt and Zetterstrom [ 121 found no essential difference between the two syndromes. Churg and Strauss
described two kinds of pathological lesion: a widespread necrotizing arteritis similar to that of polyarteriris nodosa, often containing eosinophils; and extravascular lesions made up of eosinophilic exudate,
fibrinoid changes in collagen, and granulomatous
nodules. Extravascular lesions were most numerous in
the heart but could be found in many other organs; the
cardiac changes were comparable grossly to those of
Loffler’s endocarditis. The granulomatous nodules observed in the muscle biopsy of Patient 3 (see Fig 5 ) are
similar to the extravascular lesions described by
Churg and Strauss [261. It seems probable that the
syndrome of Churg and Strauss represents a segment
of HES in which necrotizing arteritis dominates the
clinical picture, producing mononeuritis multiplex as
the principal neurological symptom.
70 Annals of N e u r o l o g y
Vol 1 No 1 January 197’
Information about prognosis in HES is contradictory. Chusid et al [ 131 found that among previously
published cases, the average survival was nine months
after diagnosis with fewer than 20% of patients remaining alive after three years. But in their own series
of 14 patients, the average survival was at least five
years and 12 patients were still alive at the time of
writing. Presumably, the previous reports were
weighted by the inclusion of autopsied cases.
The response to treatment with corticosteroids has
been inconsistent, varying from failure in many cases
(as in our Patient 1) to partial or complete remission of
symptoms (as in o u r other 2 patients). In those patients in whom the hematological findings strongly
suggested the diagnosis of eosinophilic leukemia, the
disease appeared to respond best to hydroxyurea
[27, 281. Of special interest is a patient described by
Ellman et a1 [I?], a young woman who developed
massive eosinophilia, subungual petechiae, and cardiac and muscle involvement after taking a sulfa drug.
Treatment with prednisone was ineffective, but
leukophoresis by means of a continuous-flow cell
separator reduced the eosinophilia and was followed
by rapid and lasting resolution of the systemic symptoms. This observation is the most convincing evidence so far that eosinophils in large numbers are
directly involved in causing tissue damage. The alternative possibility is that eosinophilia occurs in response to certain processes causing tissue damage
(such as antibody-antigen reactions) but is not directly
responsible for the damage. Although some evidence
seems to favor the former explanation [29], it is
noteworthy that in 1 of our patients (No. l ) , endomyocarditis progressed for many months after eosinophilia had disappeared from the blood and after the
rnyositic infiltrate had lost its eosinophilic character.
Very little is known about the role of eosinophils in
immediate hypersensitivity [ 301, and there is no direct
evidence that eosinophils contribute to tissue damage
in any disease process of which eosinophilia is a feature [311. Some authors, in fact, have suggested that
the principal function of eosinophils is to regulate or
limit allergic reactions [30]. Nevertheless, it seems
appropriate to consider treating patients who d o not
respond to corticosteroids with leukophoresis [ 171 or
with the monospecific antiserum against eosinophils
recently developed by Mahmoud et a1 [32].
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71
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