Download density (OD), measured at 490nm with an ELISA

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Letters to the Editor
density (OD), measured at 490 nm with an ELISA
reader, demonstrated that the patient’s serum reacted
to elastase (OD 0.852) and cathepsin G (OD 0.79) but
not to human albumin (OD 0.11). The control sera did
not show any reactivity (OD 0.1 for elastase, 0.09 for
cathepsin G, 0.08 for albumin). Thus, immune complexes consisting of autoantibodies and neutrophil proteases may exist in patients with a1-antitrypsin deficiency
and participate in inflammatory processes (although a
direct demonstration of their pathogenic role in our
patient was not achieved because of the ethical impossibility of obtaining a repeat biopsy specimen). The relevance of neutrophil infiltration of the dermis to the
induction of a1-antitrypsin deficiency panniculitis has
been demonstrated already [8], as has the regression of
panniculitis after the recovery of normal a1-antitrypsin
levels due to liver transplantation [9]. Together, these
findings suggest that both the direct enzymatic activity
of released proteases and CIC induced by autoimmune
reactions may generate panniculitis in the presence of
a1-antitrypsin deficiency. Furthermore, they make it
possible to hypothesize that a genetic deficiency causing
altered homeostasis between interacting molecules might
favour the onset of autoimmune reactions, perhaps due
to the breakdown of idiotypic control.
G. F, P. C, P. B, L. B,
F. I
Department of Internal Medicine, University of Genoa,
Italy
Accepted 11 April 2000
Correspondence to: F. Indiveri, Dipartimento di
Medicina Interna, Università di Genova, viale Benedetto
XV n.6, 16132 Genova, Italy.
1. Venge P, Bergstrand H, Håkansson L. Neutrophils and eosinophils.
In: Kelley WN, Harris ED Jr, Ruddy S, Sledge CB, eds. Textbook
of rheumatology, ed. 4. Philadelphia: W.B. Saunders Company,
1993:269–85.
2. Eriksson S. Alpha-1-antitrypsin deficiency: Lessons learned from
the bedside to the gene and back. Chest 1989;95:181–9.
3. Cox DW. a1-Antitrypsin deficiency. In: Scriber CR, Beaudet AL,
Sly WS, Valle D, eds. The metabolic basis of inherited disease, ed.
6. New York: McGraw-Hill, 1989:2409–37.
4. Smith KC, Pittelkow MR, Su WP. Panniculitis associated with
severe a1-antitrypsin deficiency: Treatment and review of the literature. Arch Dermatol 1987;123:1655–61.
5. Zanetti M. Idiotypy and the idiotype network in autoimmunity.
In: Bona CA, Siminovitch KA, Zanetti M, Theofilopoulos AN,
eds. The molecular pathology of autoimmune diseases. Chur,
Switzerland: Harwood, 1993:209–28.
6. Segelmark M, Elzouki AN, Wieslander J, Eriksson S. The PiZ
gene of alpha 1-antitrypsin as a determinant of outcome in
PR3-ANCA-positive vasculitis. Kidney Int 1995;48:844–50.
7. Esnault VL, Audrain MA, Sesboue R. Alpha-1-antitrypsin phenotyping in ANCA-associated diseases: one of several arguments for
protease/antiprotease imbalance in systemic vasculitis. Exp Clin
Immunogenet 1997;14:206–13.
8. Geller JD, Su WP. A subtle clue to the histopathologic diagnosis
of early alpha 1-antitrypsin deficiency panniculitis. J Am Acad
Dermatol 1994;31:241–5.
9. O’Riordan K, Blei A, Rao MS, Abecassis M. Alpha 1-antitrypsin
deficiency-associated panniculitis: resolution with intravenous
alpha 1-antitrypsin administration and liver transplantation.
Transplantation 1997;63:480–2.
Rheumatology 2000;39:1290–1292
Aseptic psoas pyomyositis and erosive discitis in a case
of calcium pyrophosphate crystal deposition disease
S, Iliopsoas abscess and pyomyositis are rare clinical
entities. Except for a few cases of carcinoma or focal
myositis mimicking abscesses [1, 2], they are infectious.
The concurrence of spondylodiscitis and bilateral psoas
abscesses is virtually pathognomonic of an infectious
process, Staphylococcus, Brucella and Mycobacterium
tuberculosis being the most likely organisms. We report
a case of calcium pyrophosphate crystal deposition
(CPPD) disease presenting with erosive spondylodiscitis
associated with an aseptic inflammatory reaction of both
psoas muscles which mimicked such an infectious
process.
A 76-yr-old woman was admitted for exacerbation of
chronic low back pain with leg weakness and repetitive
falls. There was a history of diabetes with discrete polyneuropathy, chronic venous insufficiency, hypertension
and mild chronic renal insufficiency. She had CPPD
disease with radiological chondrocalcinosis at the wrists,
knees and lumbar spine. Two years previously, a L2–L3
erosive discitis associated with a herniated disc had been
treated conservatively.
On admission, she complained of some morning stiffness and occasional night sweats and fever, but denied
any weight loss, recent surgical procedures and urogenital or gastrointestinal problems. Examination demonstrated severe limitation of lumbar spine mobility
with muscle spasms and tenderness of the paralumbar
region. There was no sign of sacroiliac joint involvement.
Light touch sensation was diminished over the anterior
thigh, and both knee and ankle jerks were absent. There
was no fever, cutaneous lesions, abdominal tenderness,
signs of cardiac involvement or any other signs of an
infectious process. The psoas sign was absent on both
sides. The white cell count was 12 000/mm3 and the
erythrocyte sedimentation rate 75 mm/h. There was mild
inflammatory anaemia and routine laboratory tests were
normal except for a moderate increase in creatinaemia.
Spine radiographs demonstrated multiple discopathies
from D9 to L4 and a previously known increased density
of the body of L2.
After 3 weeks of standard conservative therapy
consisting of analgesia, rest and non-steroidal antiinflammatory drugs (NSAIDs), she presented an exacerbation of the weakness of the quadriceps muscle. A
magnetic resonance imaging (MRI ) study demonstrated
spondylodiscitis of the L2–L3 space infiltrating the
vertebral bodies from L1 to L3. An image compatible
with an abscess or intradural sequester as well as cellulitis and myositis of both psoas muscles with enhanced
gadolinium uptake evoking abscesses were further
demonstrated ( Fig. 1).
Blood cultures, a Mantoux skin test and brucella
serology were negative. A percutaneous CT-guided
needle biopsy was performed. No organism was demonstrated on direct examination, and cultures for both
Letters to the Editor
1291
aerobic and anaerobic bacteria and for Mycobacterium,
brucella and fungi remained negative. Histology revealed
only fibro-adipose and chronic inflammatory tissues.
Because of the strong suspicion of an infectious origin,
an L2 hemilaminectomy with psoas drainage was performed. At no time before, during or after operation
were antibiotics administered. Tissue samples were sent
for culture and histology. No organism was demonstrated on direct examination and the cultures remained
negative. Histology demonstrated necrotic cartilaginous
and osseous tissues, with a severe lymphoplasmocytic
inflammatory reaction. Some giant cells, but no granulomas, were observed ( Fig. 2). The post-operative course
was favourable without antibiotic treatment, with the
disappearance of pain and neurological deficits. The
patient was discharged after 1 month and at 6 months
still had no complaints.
Iliopsoas abscess and pyomyositis are rare clinical
entities, but they are nearly always infectious. Over the
past century, the cause of this disease has changed.
Mycobacterium tuberculosis was responsible for most
cases initially, but after M. tuberculosis had been controlled as a pathogen the predominant cause became
secondary iliopsoas abscess by direct extension of an
adjacent infection [3]. Gastrointestinal tract diseases,
particularly Crohn’s disease, became the most common
cause of psoas abscess [3]. Nowadays, primary iliopsoas
abscess is increasingly related to intravenous drug abuse
and the large number of HIV-infected patients [4].
Primary iliopsoas abscess occurs primarily in young
males, but secondary abscesses are observed in a somewhat older age group. The local anatomy of the psoas
muscle explains the various causes of secondary abscesses, as this muscle is in contact with digestive, urogenital and osseous structures [5]. The source of more than
80% of secondary iliopsoas abscesses is a gastrointestinal
tract perforation [resulting from inflammatory bowel
disease (in particular severe Crohn’s disease), appendicitis, diverticulitis, carcinoma, or others] or a primary
F. 1. MRI of the lumbar spine. Gadolinium-enhanced
T -weighted images. (A) Sagittal section showing L2–L3 ero1
sive discitis with narrowing and inflammation of the disc space
with fluid (arrow) and marrow oedema extending from L1 to
L3. (B) Coronal section showing discitis with intradural free
fragment or abscess (arrow) and target lesion of the psoas
muscle with an irregular thickened wall of increased signal
surrounding a central hyposignal (arrowheads) evoking an
abscess.
F. 2. Section of a surgical biopsy stained with haematoxylin
and eosin, showing non-specific inflammatory reaction with
lymphoplasmocytic reaction, necrotic cartilage (black arrows)
and giant cells without granuloma formation (white arrows).
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Letters to the Editor
bone infection within the spine, the ileum or the sacroiliac joints, as initially appeared to be the case in our
patient [3, 5, 6 ].
Clinical diagnosis of chondrocalcinosis can be very
difficult, as signs and symptoms are not specific and can
mimic simple osteoarthritis and also rheumatoid arthritis. Most cases involve the pelvis and the extremities,
but spine involvement is well recognized [7–10]. In most
cases, chondrocalcinosis of the spine will be asymptomatic, with observation of silent radiographic disc
degeneration. Rarely, acute and chronic chondrocalcinosis may be symptomatic and produce destructive
lesions of the vertebral bodies very similar to those
observed with infections or ankylosing spondylitis.
Erosions may then proceed to secondary instability and
pseudospondylolisthesis. Such spondylitis complicates
CPPD disease in about 5–7.5% of cases [8, 11].
Myelopathy, with medullar compression secondary to
nodular deposition of CPPD crystals in the ligamentum
flavum, has also been reported [12]. Finally, involvement
of the nucleus pulposus with calcification has been
demonstrated at autopsy as well as at surgery. It can
produce an intraspinal mass with acute radiculopathy,
as seen in our case [7].
Radiographically, asymptomatic calcification of the
intervertebral disc is observed in up to 30% of patients
with systemic chondrocalcinosis, frequently at the
L2–L3 level [10]. MRI studies of CPPD involvement of
the spine have not been performed to our knowledge,
but there should be a low signal intensity on all
sequences, a finding consistent with calcification, as
opposed to infectious spondylodiscitis, which has a low
signal on T -weighted images but a hypersignal on
1
T -weighted images [13]. Definitive diagnosis is usually
2
made by demonstration of the crystals, but they can be
absent in destructive or erosive lesions [14]. Rest and
NSAIDs are the mainstay of treatment, but decompression surgery has been necessary in selected cases with
neurological deficit [15].
Thus, while crystal-related diseases are known to be
a cause of erosive discitis, and had actually been recognized 2 yr previously in our patient, the association of
myositis and fasciitis of both psoas muscles with erosive
discitis was regarded as pathognomonic of an infectious
process. All cultures, even from surgical biopsies, and
serologies were negative, arguing against such an infectious process. Biopsies demonstrated an inflammatory
infiltrate with giant cells but without granulomas. No
calcium pyrophosphate crystals were demonstrated, but
this description is compatible with previous observations
in peripheral destructive CPPD arthropathy [14]. The
aseptic causation is further supported by the favourable
evolution without antibiotic treatment.
To our knowledge, this is the first observation of
pseudo-psoas abscesses associated with erosive discitis
in a case of CPPD disease. Now that imaging techniques
such as CT and MRI are becoming increasingly prevalent, we believe it to be important to recognize that
CPPD spine disease can be accompanied by a severe
inflammatory reaction of the psoas muscles which can
mimic abscess images. This should avoid unnecessary,
costly and aggressive investigations to exclude an
infectious process.
We would like to thank Dr Guillou, Department
of Pathology, for the histological photography and
Dr Landry, Department of Radiology, for the MRI
examination.
J. D, R.-F. S, J.-C. G
Service de Rhumatologie, Médecine Physique et
Réhabilitation, Centre Hospitalier Universitaire Vaudois,
CHUV 1011 Lausanne, Switzerland
Accepted 11 April 2000
Correspondence to: J. Dudler, Service de
Rhumatologie, CHUV 1011 Lausanne, Switzerland.
1. Miyata Y, Fujii Y, Kitade K, Hara M. [A case of psoas abscess
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4. Santaella RO, Fishman EK, Lipsett PA. Primary vs secondary
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5. Simons GW, Sty JR, Starshak RJ. Retroperitoneal and retrofascial
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6. Leu SY, Leonard MB, Beart RW Jr, Dozois RR. Psoas abscess:
changing patterns of diagnosis and etiology. Dis Colon Rectum
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7. Souchon JF, Graber-Duvernay B, Gras JP. Les manifestations
rachidiennes de la chondrocalcinose. A propos d’une série de 80
observations comparée à une série appariée de sujets porteurs
d’arthrose vertébrale. Rhumatologie 1983;6:269–78.
8. Villiaumey J, Avouac B, Charlot J. L’atteinte du rachis au cours
de la chondrocalcinose articulaire. Sem Hop Paris 1981;57:1571–4.
9. Resnick D, Pineda C. Vertebral involvement in calcium pyrophosphate dihydrate crystal deposition disease. Radiographic–
pathological correlation. Radiology 1984;153:55–60.
10. Salcman M, Khan A, Symonds DA. Calcium pyrophosphate
arthropathy of the spine: case report and review of the literature.
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80 observations. Rev Rhum 1980;47:337–43.
12. Ferrer-Roca O, Brancos MA, Franco M, Sabate C, Querol JR.
Massive articular chondrocalcinosis. Its occurrence with calcium
pyrophosphate crystal deposits in nucleus pulposus. Arch Pathol
Lab Med 1982;106:352–4.
13. Frocrain L, Duvauferrier R, Chalès G, Martin A, Moisan A,
Ramée A et al. Une nouvelle méthode diagnostique de la spondylodiscite. L’imagerie par résonance magnétique. J Radiol 1987;68:
373–80.
14. Lagier R. L’approche anatomo-pathologique du concept de chondrocalcinose articulaire. Rhumatologie 1981;9:421–37.
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Rhumatologie 1980;10:247–9.
Rheumatology 2000;39:1292–1294
Who is referred for specialist rheumatology care?
S, Although referral for specialist advice in secondary
care rheumatology departments is a key determinant of