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Relapsing polychondritis with aortic involvement.

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Relapsing Polychondritis with Aortic Involvement
Duncan S. Owen Jr, Robert lrby and Elam Toone
A 35-year-old female with relapsing polychondritis and aortic insufficiency is
presented. Cases previously reported are reviewed. Aortic insufficiency appears
to be fairly common in cases of relapsing polychondritis.
Very little attention had been directed to These symptoms were associated with fever and
relapsing polychondritis until 1958, when pain in the cervical and thoracic spine. Prednisotie
therapy was instituted with benefit, but 20 mg daily
Dr. William Bean reviewed 8 cases from was
required continuously for relief of symptoms. A
the world’s literature and reported 1 of his brother, age 37, is reported to have ankylosing
own (1). I n this disorder, cartilage inflam- spondylitis.
Initial examination revealed a slightly obese
mation is the predominating feature, but
there are an increasing number of reports female with a mild cushingoid appearance who was
afebrile. T h e blood pressure was 110/70. Pertinent
with aortic involvement (2-4). The case and significant findings revealed a left ear which
reported below is that of a young female was deformed and floppy, a saddle deformity of the
with a classical case of relapsing polychon- nose (Fig. 1 ) , and a very mild episcleritis. T h e
dritis who subsequently developed aortic remainder of the examination, including careful
insufficiency 20 months after the onset of cardiac examination, was negative.
Laboratory studies revealed an erythrocyte sediher symptoms.
mentation rate of 25 mm/hr (Wintrobe-corrected) .
JT, a 35-year-old Caucasian female, was originally
seen on Apr 17, 1968, for an evaluation of relapsing
polychondritis which had been diagnosed previously. The patient had been in good health until Jan
1967, when severe episcleritis and anterior a s t o chondral pain developed requiring systemic corticosteroids for relief. Pain in the regioii of the
thyroid cartilage developed in May 1967, and later,
in Oct, nose and left ear pain and swelling ensued.
From the Division of Connective Tissue Disease,
Department of Medicine, Medical College of Virginia, School of Medicine, Virginia Commonwealth
University, Richmond, Virginia.
Supported in part by Research Grant AM 04549
and Training Grant T I AM5056 US Public Health
Publication No. 23 from the Charles W. Thomas
Arthritis Fund, Medical College of Virginia.
Address for reprint requests: Duncan S. Owen,
Jr, MD, 1200 East Broad Street, Richmond, Virginia 23219.
Submitted for publication July 7, 1969; accepted
Aug 25, 1970.
The white blood count was 18,2OO/cu mm with a
normal differential. Normal or negative values weIe
noted for the following: hemoglobin, urinalysis,
VDRL, LE cell preparation, serum uric acid, and
rheumatoid factor (latex flocculation and sensitized
human cell tests).
One week after the initial consultation, severe left
episcleritis developed. Prednisone was increased to
60 mg/day, and a trial of indomethacin. was
instituted. T h e episcleritis, which was very difficult
to control initially, was much improved 6 weeks
later. T h e dose of prednisone was subsequently
reduced to 15 mg/day, and indomethacin 150
mg/day was continued.
T h e patient complained of mild dyspnea on
exertion on Sept 4, 1968. At that time, it was
noted that her pulse was 96/min and regular, and
her blood pressure 115/55-0. A grade II/VI highpitched basilar decrescendo diastolic murmur was
noted which radiated along the left sternal border,
and a grade 1I/VI harsh ejection systolic murmur
which transmitted faintly to the neck vessels was
also heard. Neither of these murmurs had been
*Indocin@,Merck Sharp Rc Dohme, West Point, Pa.
M h f M s and Rheumatism, Vol. 13, No. 6 (November-December 1970)
An extensive review of 49 cases of relapsing polychondritis was reported in 1966
(5). In this review, it was noted that a case
of aortic insufficiency associated with relapsing polychondritis had been reported from
Japan. Pearson later reported 2 cases with
aortic involvement (2).
One of Pearson’s cases was a 33-year-old
male who developed severe aortic insufficiency and congestive heart failure 7 years
after the onset of polychondritis. At operation, normal aortic valve leaflets were
noted, but the aortic ring and initial 1.5 cm
of the ascending aorta were markedly dilated and thin. T h e other case was a
26-year-old male who developed aortic insufficiency 3 years after the onset of polychondritis. At surgery, again noted were
normal aortic valves and a dilated ring,
and there was also a n aneurysm of the
ascending aorta. Pathologic studies revealed aortic changes quite similar to those
seen in syphilis, but no endothelial prolifFig 1. Polychondritis showing deformed ear and nose.
eration was present. Only a small loss of:
neutral or acidic polysaccharides was
noted, but there was marked deple,tion of
present on the initial examination. There was no
evidence of active polychondritis.
amounts of PAS-staining material were
T h e patient was then admitted to the Medical
noted. Pearson mentioned 2 other patients
College of Virginia Hospital for further evaluation.
Laboratory studies revealed that the hemogIobin
with aortic involvement who had been rehad fallen to 9.1 g/100 ml, and the erythrocyte
ported to him verbally, plus the case from
sedimentation rate had risen to 56 mm/hr, (winJapan (2). Self, reported a case of relapstrobe-corrected) . Radiographic heart studies reing polychondritis associated with cystic
vealed widening of the aortic arch with prominence
of the ascending and descending aorta (Fig. 2 ) . medial necrosis of the aorta (4).Hainer, in
Blood cultures and further LE cell preparations
1969, reported a case in which a dissecting
were negative.
aneurysm developed (3).
Subsequent course. Digitalization was instituted
It is interesting to note that Thomas and
and has been continued. Prednisone dosage was
McCluskey induced cartilage dissolution in
changed to 40 mg in single dose form every other
rabbit ears b y the intravenous administraday. A subsequent attempt to reduce prednisone to
30 mg resulted in moderate tenderness in the
tion of papain, and a loss of cartilage
thyroid cartilage. T h e ,patient was last seen in June
metacliromasia, somewhat similar to that
1969, was essentially asymptomatic, and is receiving
seen in polychondritis, was noted (6, 7).
prednisone, 35 mg every other day, and digitalis.
Later, Tsaltas noted aortic changes in rabT h e cardiac findings are unchanged, but the cushbits 3 to 6 weeks after administering papain
ingoid appearance has disappeared.
Arthritis and Rheumatism, Vol. 13, No. 6 (November-December 1970)
Fig 2. Chest x-ray film of patient with polychondritis showing widening of the aortic arch with prominence
of the ascending and descending aorta.
intravenously. Alterations were noted in
the media, and cartilage and bone developed in this area (8).
1. Bean WB, Drevets C, Chapman JS: Chronic
atrophic polychondritis. Medicine 37:353,
2. Pearson CM, Kroening R, Verity MA, et
al: Aortic insufficiency and aortic aneu-
rysm in relapsing polychondritis. Trans
Ass Amer Physicians 80:71, 1967
3. Hainer JW, Hamilton GW: Aortic abnormalities in relapsing polychondritis:
report of a case with dissecting aortic aneurysm. New Eng J Med 280:1166. 1969
4. Self J, Hammarsten JF, Lyne B, Peterson
DA: Relapsing polychondritis. Arch Intern Med 120:109, 1967
5. Dolan DL, Lemmon GB Jr, Teitelbaum
SL: Relapsing polychondritis: analytical
Arthritis and Rheumatism, Vol. 13, No. 6 (November-December 1970)
speculate further that the common denominator
between cartilage, aorta and the eye (in the
aforementioned review, 60% had episcleritis and/or
conjunctivitis) is a chemical constitutent of these
connective tissues, against which host antibody is
directed. The protein moiety of the proteinpolysaccharide complexes in these tissues might ,be
the target of such an immunologic attack. Of the
various organs affected, cartilage may be most
vulnerable because its structure depends so heavily
on the integrity of the chondromucoprotein complex.
T h e above speculationtemphasizing immunologic mediation of cartilage injury in polychondritiscan be approached by direct experimentation. Aside
from the attempt with the technic of immunofluorescence, the reviewer is not aware of studies
Charles L. Christian, M D , New York, New York: designed to show reactions between sera of patients
with the syndrome and extracts, or more purified
This case report re-emphasizes the interesting assocomponents of cartilage. If the patient is at hand,
ciation between polychondritis and aortic pathology. As indicated in the discussion, the literature skin reactions to cartilage products could be
refers to seven previous cases with this combina- studied. The sera and urine, if analyzed, might
demonstrate increased excretion of sulfated mucotion.
The authors comment on the pathologic similari- polysaccharides similar to that observed with papain-induced cartilage lysis ( 6 ) . [A reported inties between polychondritis and the experimental
in urine mucopolysaccharides (carbazole
model induced #by papain-the essential difference
reaction) during active polychondritis was not
being the absence of inflammation with the latter
controlled for nonspecific inflammatory effect.] (7)
(1) *
Fortunately most patients exhibit intermittent
The loss of basaphilic staining of affected cartiactivity
of the disease and exacerbations usually
lage is consistent with the thesis that a proteolytic
respond to treatment with corticosteroids. The
process is involved. Accepting this premise, the
question-Does such therapy early in the course of
questions are: Is there a systemic elaboration of
the disease lessen the likelihood of visceral involveenzyme analogous to the papain model? Are cellular
cathepsins in situ activated secondarily by chemical ment?-will be difficult to answer because of the
relative rarity of the syndrome.
or immunologic events? There are two exDerimental counterparts of the latter: a) vitamin A excess,
which mimics the lesions induced by paDain (2).
and b) the model of complement-dependent cartilage lysis in vitro which is mediated b y anti-cell
1. Thomas L: Reversible collapse of rabbit
membrane antibody (3.4). An attempt to demonears after intravenous papain, and prevenstrate anti-cartilaee antibodies (immunofluorestion of recovery by cortisone. J Exp Med
cence) in 2 cases of polychondritis was inconclusive
104:245, 1956
hecause of nonspecific fluorescence ( 5 ) . The possi2.
L, McCluskey R T , Potter JL,
bility of an immunologic basis for polychondritis
et al: Comparison of the effects of papain
gains some support from the observed disease
and vitamin A o n cartilage. I. T h e effects
associations. From a review of 51 cases in the
literature, there were 9 with concomitant rheumain rabbits. J
- Exp
- Med 111:705, 1960
toid arthritis, systemic lupus erythematosus, or
3. Fell HR, Coombs RRA, Dingle JT: T h e
Sjogren's syndrome-illnesses with acknowledged
breakdown of embryonic (chick) cartilage
autoimmune associations ( 5 ) . This discussant has
a n d bone cultivated i n the presence of
recognized only 2 cases of polychondritis from
complement-sufficient antiserum. I. Morpersonal experience, one of whom had typical
phological changes, their reversibility and
systemic lupus erythematosus.
inhibition. I n t Arch Allergy 30:146, 1966
On the basis of the foregoing. it is attractive to
literature review a n d studies on pathogenesis. Amer J Med 41:285, 1966
6. Thomas L: Reversible collapse of rabbit
ears after intravenous papain, a n d prevention of recovery by cortisone. J Exp Med
104245, 1956
7. McCluskey RT, Thomas L: Removal of
cartilage matrix i n vivo, by papain. J Exp
Med 108:371, 1958
8 Tsaltas TT: Metaplasia of aortic connective tissue to cartilage and bone induced
by the intravenous injection of papain.
Nature 196:1006, 1962
Arthritis and Rheumatism, Vol. 13, No. 6 (November-December 1970)
4. Lachmann PJ, Coombs RKA, Fell HB,
et al: T h e breakdown of embryonic (chick)
cartilage and bone cultivated in the presence of complemen t-sufficient antiserum.
111. Immunological analysis. Int Arch Allergy 36:469, 1969
5. Dolan DL, Lemnion GB Jr, Teitelbaum
SL: Relapsing polychondritis. Analytical
literature review and studies on patho-
genesis. Amer J Med 41285, 1966
6. Bryant JH, Leder IG, Stetten D Jr: The
release of chondroitin sulfate from rabbit
cartilage following the intravenous injection of crude papain. Arch Biochem Riophys 76:122, 1958
7. Kaye RL, Sones D.4: Kelapsing polychondritis: clinical and pathologic features in
fourteen cases. Ann Int Med 60:653, 1964
Mhritis and Rheumatism, Vol. 13, No. 6 (November-December 1970)
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involvement, relapsing, polychondritis, aortic
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