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Joint changes with progressive systemic sclerosis.

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me bhropathy of progressive Systemic sclerosis
To the Editor:
We would like to comment on the report by Dr.
Blocka and his colleagues (1) on the arthropathy of progressive systemic sclerosis (PSS).
First, according to the American Rheumatism Association (ARA) criteria, scleroderma proximal to the metacarpophalangeal joints (proximal scleroderma) is the major
diagnostic criterion for PSS (2). However, this term does not
distinguish between diffuse skin involvement and acrosclerosis (sclerodermatous involvement limited to distal extremities but which sometimes involves distal forearm and face),
which may occur in patients with CREST (calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly,
telangiectasis). According to Blocka et al, proximal scleroderma as defined by the ARA was used as a synonym for
diffuse scleroderma. This misrepresentation could cause
physicians to misgroup patients clinically, which could affect
correlations made between radiologic or serologic findings
and clinical subsets.
Second, because an erosive arthropathy was an
uncommon finding in the CREST patients, W e would like to
report another case. A 68-year-old woman developed a
seronegative polyarthritis in 1972, and she was diagnosed as
having rheumatoid arthritis. However, she had long-standing
Raynaud's phenomenon and developed sclerodactyly in
1976. Subsequently, she developed telangiectasia and subcutaneous calcification in her hands. On an electrocardiogram,
a left bundle branch block was shown, and results of a
barium swallow were indicative of PSS. On a chest radiograph, bibasilar fibrosis was shown, and results of lung
function tests were indicative of a decreased carbon monox-
ide transfer. Her main complaint throughout, however, has
been arthritis, mainly affecting the hands and wrists. On a
hand radiograph (Figure l), progressive erosion of the left
ulnar styloid is shown. There are also margin4 erosions of
the proximal interphalangeal joints but no lesions in other
joints. Anticentromere antibodies were detected by indirect
immunofluorescence with HEp-2 cells (3). These antibodies
appear to have a high specificity for CREST (4,5). Results of
tests for rheumatoid factor remained negative.
For this patient who had clinical and serologic features of the CREST variant of PSS, there were definite
erosive changes in her wrist radiographs. Although joint
involvement seems to be less frequent in patients with this
variant, it may be indistinguishable from patients with diffuse PSS.
Luis J. C a t o ~ oMD
Peter J. Maddison, MD, MRCP
Royal National Hospital
for Rheumatic Diseases
Bath BAl IRL, England
1. Blocka KLN, Bassett LW, Furst D, Clements P, Paulus H: The
arthropathy of advanced progressive systemic sclerosis: a radiographic survey. Arthritis Rheum 24374-884, 1981
2. Subcommittee for Scleroderma Criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee: Preliminary criteria for the classification of systemic
sclerosis (scleroderma). Arthritis Rheum 23:581-590, 1980
3. Moroi Y ,Peebles C, Fritzler M,Steigerwald J, Tan E: Autoantibodies to centromere (kinetochore) in scleroderma sera. Proc
Natl Acad Sci USA 77:1627-1631, 1980
4. Tan EM, Rodnan GP, Garcia I, Moroi Y ,Fritzler M, Peebles C:
Diversity of antinuclear antibodies in progressive systemic sclerosis. Arthritis Rheum 23:617-625, 1980
5. Fritzler MJ, Kinsella TD, Garbutt E: The CREST syndrome: a
distinct serologic entity with anticentromere antibodies. Am J
Med 69:52&526, 1980
Joint changes with progressive systemic sclerosis
Figure 1. Progressive erosion of left ulnar styloid is shown on
radiograph of left wrist (1979).
To the Editor:
We read with interest the article regarding joint
changes with progressive systemic sclerosis by Blocka et al
(1). Recently we have been involved in the care of a woman
with CREST (calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, telangiectasia) syndrome
and severe erosive arthropathy of both first metatarsophalangeal joints.
A 39-year-old woman had a 5-year history of scleroderma; we began to treat her after she had had episodic pain
in both feet for 1 year. The pain had become disabling by the
time we first saw her. She had been treated with prednisone,
10 mg daily for the past 4 years, but not with any other
medications. In the results of the physical examination, both
characteristic facial and finger changes of scleroderma and
marked ankylosis of both wrists were shown. There were
swelling, erythema, and tenderness of the first metatarsophalangeal (MTP) joints of her feet, and notable shortening
of both first toes, 3-4 cm on the left and 1-2 cm on the right.
On roentgenograms of the wrists, bilateral destruction of the
carpal bones with marked soft tissue calcification was
shown; bilateral erosion and destruction of the first distal
metatarsals and proximal phalanges, again accompanied by
soft tissue calcification, were shown on roentgenograms of
the feet (Figures 1 and 2). Evaluations of the cardiopulmonary, renal, and gastrointestinal systems failed to document
more widespread involvement of her disease.
The patient underwent resection arthroplasty of the
left first MTP joint. Total destruction of the joint surface and
marked calcification of the capsular tissues were found, but
there was no evidence of synovial proliferation. In the
results of tissue pathology, severe scarring and dystrophic
calcification of the soft tissues and extensive bone necrosis
with fibrosis of the intratrabecular bony spaces consistent
with chronic osteomyelitis were shown, although intraoperative cultures of this joint grew few Staphylococcus aureus
and a right first MTP joint aspirate yielded no growth.
In the recent literature, the reporting on arthropathy
and scleroderma (either as progressive systemic sclerosis or
CREST) has focused primarily on hand involvement (2-5),
although temporomandibular joint (6) and femoral head (7)
destruction have also been reported. Only occasionally has
metatarsal involvement been mentioned (8). Blocka et a1 (1)
mention 1 patient with a “pencil-in-cup” deformity, and
Karten (9) described severe destruction of the MTPjoints in
a patient with CREST and neuropathic arthropathy .
Our patient is unique in the remarkable progressive
destruction of the involved MTP joints without apparent
etiology. Her case is complicated by the chronic use of
steroids, although clinically and pathologically her changes
Figure 1. Radiographs of both feet. Note the marked destruction
and soft tissue calcification of the first metatarsophalangeal joints.
Figure 2. Radiographs of the hands. Note the carpal decompression
and soft tissue calcification.
are not indicative of aseptic necrosis. Further, although
there was evidence of a possible septic process in a single
joint, such a process would not account for the symmetric
nature of her disease. The destruction seems to be related to
her primary disease and is most likely related to the vascular
changes that occur with scleroderma.
We propose that the erosive arthropathy of scleroderrna, although unusual, is actually more commonplace
than previously recognized. The increased frequency of
recent reports confirms this conclusion. The arthropathy
may be occurring even in asymptomatic areas, and by
routine radiographs, the physician may document unsuspected joint destruction.
Silvia Rouzaud, MD
Arnold L. Katz, MD
University of Oklahoma
Tulsa Medical College
Tulsa, Oklahoma
1. Blocka KLN, Bassett LW, Furst DE, Clements PJ, Paulus HE:
The arthropathy of advanced progressive systemic sclerosis.
Arthritis Rheum 242374-884, 1981
2. Love11 CR, Jayson MIV: Joint involvement in systemic sclerosis.
Scand J Rheumatol 8:154-160, 1979
3. Brower AC, Resnick D, Karlin C, Piper S: Unusual articular
changes of the hand in scleroderma. Skeletal Radio1 4:119-123,
4. Resnick D, Greenway G, Vint VC, Robinson CA, Piper S:
Selective involvement of the first carpometacarpal joint in scleroderrna. Am J Roentgen01 131:283-286, 1978
5. Wild W, Beetham WP Jr: Erosive arthropathy in systemic
scleroderma. JAMA 232:511-512, 1975
6. Osial TA Jr, Avakian A, Sassouni V, Aganval A, Medsger TA Jr,
Rodnan GP: Resorption of the mandibular condyles and coronoid
processes in progressive systemic sclerosis. Arthritis Rheum
24~129-733, 1981
7. Wilde AH, Mankin HJ, Rodnan GP: Avascular necrosis of the
femoral head in scleroderma. Arthritis Rheum 13:445-447, 1970
8. Rodnan GP: The nature of joint involvement in progressive
systemic sclerosis. Ann Intern Med 56:422-439, 1962
9. Karten I: CREST Syndrome and “neuropathic” arthropathy.
Arthritis Rheum 12:636-638, 1969
“Rugger jersey” and osteopetrosis
To the Editor:
The reintroduction of radiologic vignettes is greatly
appreciated. Dr. Resnick has picked an interesting topic for
the first vignette-the “rugger jersey” vertebral body (I). I
very much enjoyed his excellent discussion of this intriguing
radiologic finding, his radiologic-pathologic correlations,
and the beautiful example of Paget’s disease and sclerosing
When I noted the title, I had initially expected a
discussion of the disease described by Albers-Schonberg in
1904: osteopetrosis ( 2 ) . Osteopetrosis (3,4) is one of a group
of disorders characterized by varying degrees of skeletal
sclerosis (5). The adult or tarda form of osteopetrosis is often
complicated by vertebral involvement. While vertebrae may
be homogeneously radiodense (3), the vertebral bodies more
commonly present with sclerosis of the vertebral end plates
producing two dense horizontal bands of bone separated by
a relatively radiolucent region (6,7) (Figure I ) .
Pathologic evaluation of vertebrae reveal similar
changes in osteopetrosis (8) and renal osteodystrophy . The
two disorders, however, can be fairly easily distinguished
because of the characteristic companion sclerotic involvement of other bones in osteosclerosis. Figure 1 , for example,
demonstrates characteristic bands of calcification along the
iliac crest.
Figure 1. Lateral view of lumbar spine. Note “ruggerjersey” spine
and bands of increased radiodensity circling the iliac crest.
Bruce M. Rothschild, MD
Veterans Administration Medical Center
North Chicago, ZL
5 . Hinkel CL: Developmental affections of the skeleton character-
1. Resnick D: The “rugger jersey” vertebral body. Arthritis Rheum
24: 1191-1 194, 1981
2. Albers-Schonberg H: Rottgenbildereiner Seltenen Knochenerkrankung. Muench Med Wschr 51:365-369, 1904
3. Seigman EL, Kilby WL: Osteopetrosis: report of a case and
review of recent literature. Am J Roent 63:865-874, 1950
4. Johnston CC Jr, Lavy N, Lord T, Vellios F, Merritt AD, Deiss
WP Jr: Osteopetrosis: a clinical, genetic, metabolic and morphologic study of the dominantly inherited benign form. Medicine
47~149-167, 1968
ized by osteosclerosis. Clin Orthop 9:85-105, 1957
6. Fairbank HA: Osteopetrosis. J Bone Joint Surg 30B:339-356,
7. Beighton P, Hamersma H, Cremin BJ: Osteopetrosis in South
Africa: the benign, lethal and intermediate forms. S Afr Med J
55:659-665, 1976
8. Whyte MP, Murphy WA, Fallon MD, Sly WS, Teitelbaum SL,
McAlister WH, Avioli LV: Osteopetrosis, renal tubular acidosis
and basal ganglia calcification in three sisters. Am J Med 69:6474. 1980
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progressive, change, joint, systemic, sclerosis
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