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Destructive 2-microglobulin amyloid arthropathy of the cervico-occipital hinge in a hemodialyzed patient.

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Destructive &-microglobulin amyloid arthropathy of
the cervico-occipital hinge in a hernodialyzed patient
To the Editor:
Osteoarticular complications represent a major concern in patients treated with long-term hemodialysis, hemofiltration, or continuous ambulatory peritoneal dialysis (1,2).
Kuntz and colleagues (3) described a destructive spondylarthropathy involving cervical, thoracic, or lumbar segments
of the spine in patients receiving long-term hemodialysis.
Sebert and colleagues (4) found amyloid infiltration of the
invertebral discs in a dialyzed patient who developed destructive spondylarthropathy of the cervical spine. These
amyloid deposits are probably composed of P,-microglobulin (p2m), which has been identified as a causative agent in
dialysis-related amyloidosis.
Radiologically demonstrable spinal lesions are predominantly located in the cervical spine, and the C S C 6 and
C M 7 discs are most frequently involved. There are no
reports of lesions of the cervico-occipital hinge.
We report herein the case of a patient receiving
hemodialysis for 16 years who developed destructive arthropathy of the cervico-occipital hinge due to amyloid deposits composed of P2m. The patient, a 42-year-old woman,
experienced a corticosteroid-resistant nephrotic syndrome
at age 1 1. The disease developed into a progressive deterioration of renal function requiring hemodialysis treatment
(using a cuprophan membrane), beginning in January 1971
and continuing twice a week for I5 years. In 1976, she
developed hyperparathyroidism, necessitating a subtotal
parathyroidectomy. In order to control resistant hypertension, a bilateral nephrectomy was performed in 1978; this
was followed by severe anemia requiring blood transfusions
every 4-6 weeks. A posttransfusional iron overload became
evident (plasma ferritin 13,200 &liter in 1986).
In 1980, articular complications became apparent for
the first time. The patient reported having shoulder and knee
pain, and had recurring bilateral carpal tunnel syndrome
(CTS) requiring 2 operations at each site, and "spring
fingers" due to flexor tendon tenosynovitis.
A histologic examination, conducted in 1985 on a
synovial tissue sample obtained during surgery for CTS,
showed a massive infiltration of amyloid deposits, which was
identified by Congo red-induced dichroism. Immunoperoxidase studies showed staining with anti-am, and the Prussian blue reaction revealed the presence of intracellular
In July 1987, neuralgia appeared, affecting the great
occipital nerve; the condition rapidly became bilateral and
severe. On examination, there was cramping of the cervical
muscles, which was aggravated by rotational movements of
the head. Radiographic studies revealed cysts of the humeral
and femoral heads, the cotyles, and the carpal bones. There
was also destructive spondylarthropathy of the C b C 7 discs,
with significant abnormalities of the cervico-occipital hinge
joint, including atlantoaxial diastasis, thickening of the soft
tissue, and basilar impression (Figure I A). Computed tomography showed destructive lesions of the lateral masses
Arthritis and Rheumatism, Vol. 33, No. 4 (April 1990)
of the atlas (Figure 1B). Magnetic resonance imaging revealed erosive changes of the top of the odontoid process,
which was surrounded by a pseudotumoral mass (Figure 1C).
The histologic study of the percutaneous biopsy
sample of this paraverterbral mass showed amyloid deposits
that were reactive with anti-am, separated by a few vessels
and pigmented cells. The Prussian blue reaction identified
the extra- and intramacrophagial deposits of hemosiderin;
considering the neurologic risks, surgery was performed in
February 1988. The cervico-occipital space was enlarged by
posterior laminectomy of the C1 and C2 discs. The dura
mater was reconstructed, and an iliac graft was inserted.
Completed disruption of the bone structure by massive
amyloid deposits was observed in the bone samples obtained. Postoperative recovery was good, and the neuralgia
After treatment with long-term hemodialysis for 17
years, the patient developed osteoarticular complications
typical of what is now known as dialysis arthropathy (1)
(usually encountered between the seventh and tenth year of
dialysis). The patient's CTS had the usual characteristics (5)
of being bilateral, recurrent after surgical decompression,
and associated with tenosynovitis of the flexor tendons.
Radiologic features were very similar to those observed in
long-term dialysis patients with juxtaarticular destruction of
the hips, shoulders, and wrists, and destructive cervical
spondylarthropathy ( C W 7 ) .
Destructive spondylarthropathy is characterized by
discovertebral erosions or destruction and disc space narrowing, without significant osteophytosis (3). The lower segment of
the cervical spine is the most frequent site, and abnormalities of
the cervico-occipital hinge are unusual. This is the first reported case of neurologic complications due to P2m amyloid
destruction of the cervico-occipital hinge.
Amyloid deposits reacting with anti-p,m were found
in all of the samples taken, which corresponds to the type of
amyloidosis identified in dialyzed patients reported in 1985
(6). The frequency with which these deposits are found in
articular samples and the fact that the joints represent a
preferential and often unique localization of the deposits
support a pathogenic role of p2m amyloidosis in dialysis
The pathogenesis of dialysis arthropathy, although
still unknown, is probably multifactorial. In fact, a group of
associated factors, including the duration of dialysis ( 9 , the
type of dialysis membrane (7), the patient's age (8), the effect
of hyperparathyroidism on the skeletal system, the presence
of apatite crystals (3), and an aluminum (9) and iron overload
(1-10). may play a role. The latter factor could be a consequence of chronic inflammation or posttransfusional hemosiderosis, as observed in our patient.
Our observations suggest that lesions of the cervicooccipital hinge should be added to the growing list of
manifestations of P2m amyloidosis. These lesions are identical to those found in rheumatoid arthritis and may lead to
the same neurologic complications. Early detection is important, but conventional radiologic surveys are not likely to
detect early geodic lesions of the atlas, the axis, or the
odontoid process. Considering the risk of cervical myelopathy, it may be useful to perform examinations that are more
likely to identify the abnormalities, especially magnetic
Figure 1. A, Sagittal radiography of the cervical spine, showing destructive lesions of the C K 7 discs, atlantoaxial diastasis,
thickening of the soft tissue, and basilar impression (arrow). B, Computed tomography of the cervico-occipital hinge; showing
destructive lesions of the lateral masses of the atlas (arrows). C, Sagittal EG 380/21 magnetic resonance image of the cervico-occipital
hinge, showing erosive changes of the top of the odontoid process, surrounded by a pseudotumoral mass (arrows).
resonance imaging of the cervico-occipital hinge, in patients
who have undergone hemodialysis for more than 7 years.
Extreme caution should be exercised if patients with lesions
of the cervico-occipital hinge need to be intubated for
general anesthesia.
Michele Kessler, MD
Patrick Netter, MD
Bruno Grignon, MD
Jean Michel Bertheau, MD
Bernadette Aymard, MD
Eric Azoulay, MD
Alain Gaucher. MD
URA CNRS 1288, CHU de Nancy
Vandoeuvre les Nancy, France
1. Brown EA. Arnold 1R. Gower PE: Complications of long term
treatment with haemodialysis. Br Med J 292:163-166, 1986
2. Cornelis F, Bardin T, Faller B, Verger C, Allouache M ,
Raymond P. Rottembourg J. Tourli@reC, Benhamou C, Noel
LH, Kuntz D: Rheumatic syndromes and P,-microglobulin
amyloidosis in patients receiving long-term peritoneal dialysis.
Arthritis Rheum 32:785-788, 1989
3. Kuntz D. Naveau B, Bardin T , Drueke T, Treves
Dryll A:
Destructive spondylarthropathy in hemodialyzed patients: a
new syndrome. Arthritis Rheum 27:369-375. 1984
4. Sebert J-L, Fardellone P, Mane A, Deramond H, Lambrey G,
Legars D, Galibert P, Smaida A, Fournier A: Destructive
spondylarthropathy in hemodialyzed patients: possible role of
amvloidosis (letter). Arthritis Rheum 29:301-303. 1986
5. Charra B. Calemard E, Uzan M, Terrat JC, Vanel'T, Laurent G:
Carpal tunnel syndrome shoulder pain and amyloid deposits in
long term haemodialysis patients. Proc Eur Dial Transplant
ASSOC21~291-295, 1985
6 . Gejyo F, Yamada T, Odani S, Nakagawa Y, Kunitomo T,
Kataoka H, Suzuki M, Himarawa Y , Shirahama T, Cohen AS,
Schmid K: A new form of amyloid protein associated with
hemodialysis was identified as p2 microglobulin. Biochem
Biophys Res Commun 129:701-706, 1985
7. Vandenbroucke J, Jadoul M, Maldague B, Huaux JP, Noel H,
van Ypersele de Strihou C: Possible role of dialysis membrane
characteristics in amyloid osteoarthropathy. Lancet 11: 12101211, 1986
8. Gaucher A, Kessler M, Netter P, Azoulay E, Pert P, Mur JM:
Dialysis arthropathy: the effect of age. J Rheumatol 15:
1880-1881, 1988
9. Netter P, Kessler M, Gaucher A, Gillet P, Delons S, Burnel D,
Benoit J , Got C: Aluminum and dialysis arthropathy. Lancet
IL886-887, 1988
10. Cary NRB, Sethi D, Brown A, Erhardt CC, Woodrow DF,
Gower PE: Dialysis arthropathy: amyloid or iron? Br Med J
293~1392-1394. 1986
Cricoarytenoid synovitis in ankylosing spondylitis
To the Editor:
The cricoarytenoid joint is a diarthrodial articulation
with a ligamentous capsule lined by synovial membrane (1).
Involvement of this joint in rheumatoid arthritis (RA) has
been well documented clinically and pathologically ( I-3),
whereas cricoarytenoid joint synovitis is an unusual feature
of ankylosing spondylitis (4-7). We present herein the clinicopathologic features of severe cricoarytenoid synovitis in a
patient with longstanding ankylosing spondylitis.
The patient, a 79-year-old white man, was diagnosed
as having ankylosing spondylitis at age 25, on the basis of
persistent back pain and stiffness. Over the next several
years, spine radiographs showed evidence of complete ankylosis at both sacroiliac joints and the entire lumbosacral,
thoracic, and cervical spines. His HLA-B27 status was
never ascertained. Four years prior to admission, the patient
noted progressive difficulty ambulating and changes in bowel
and bladder habits. He underwent a cervical laminectomy
for spinal stenosis; however, neurologic symptoms persisted.
Two months prior to admission, he developed dysphagia and a productive cough, and he noted a 20-lb weight
loss. Results of a chest radiograph, endoscopy of the upper
gastrointestinal tract, and a barium swallow were reported as
normal. A few weeks later, he developed laryngeal stridor
and hypotension and was admitted to another hospital. A
laryngoscopy revealed arytenoid edema and right vocal cord
paralysis. He was treated with high doses of intravenous
corticosteroids, with resolution of his stridor. Steroids were
subsequently tapered, and the stridor recurred, along with a
new right lower lobe infiltrate. A laryngoscopy revealed
bilateral vocal cord paralysis and a posterior cricoid ulcerative lesion extending to the hypopharynx. A thyrocricoid
tracheostomy was performed, which relieved the airways
obstruction. A few days later, the patient developed hypotension, oliguria, lethargy, and an elevation of his white
blood cell count to 18,000 cells/mm3. Despite the initiation of
broad-spectrum antibiotic and antifungal therapy, his condition continued to deteriorate, and he died shortly thereafter.
Figure 1. Low power photomicroscopic view of the left cricoarytenoid joint. A small amount of articular cartilage remains in the
arytenoid area (seen on the right);the articular surface of the cricoid
cartilage is denuded (seen on the left). Both cartilages show extensive calcification (arrowheads). The joint space is filled with dark
hemorrhagic material, fibrin, and inflammatory cells (original magnification x 10).
At autopsy, there was evidence of a severe chronic,
active cricoarytenoiditis with inflammatory obliteration of
the cricoarytenoid joints bilaterally. The intense inflammatory reaction surrounding the laryngeal cartilages in the
region of the cricoarytenoid joints led to ulceration of the
overlying laryngeal and esophageal mucosae (Figure 1). No
tumor was identified, and special stains for organisms were
negative. An acute necrotizing aspiration pneumonia involving the right upper and middle lobes was also identified and
was the immediate cause of death.
A compilation of previously reported cases of cricoarytenoid synovitis in association with ankylosing
spondylitis (4-7) indicates that patients had longstanding
disease (mean 26.5 years) with extensive spinal ankylosis
including the cervical spine. Cricoarytenoid synovitis resulted in some degree of airways compromise in 5 of 6
patients. Initial manifestations included dyspnea and hoarseness, stridor, and throat pain. Three patients developed
acute respiratory failure, and 2 patients aspirated.
The management of cricoarytenoid synovitis in ankylosing spondylitis included tracheostomy (3 of 6 patients),
arytenoidectomy ( 1 of 6), and the use of corticosteroids
either orally or intraarticularly. Bienenstock et al (1) correlated the results of cricoarytenoid joint laryngoscopic examination with clinical symptoms in patients with RA. When
both cricoarytenoid joints were involved, bowing of the
vocal cords was seen during the inspiratory phase of respiration. Chronic unilateral disease resulted in slight hoarseness andlor dyspnea on exertion, whereas dyspnea would
predominate with bilateral cricoarytenoid joint disease. Fixation of the vocal cords in adduction resulted in hoarseness
or aphonia.
The pathologic findings in our patient, of extensive
inflammatory destruction of the cricoarytenoid joints with
“spillover” of the process into adjacent tissues such as the
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microglobulin, occipital, arthropathy, patients, destruction, hinged, cervico, hemodialyzed, amyloid
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