Skeletal Findings in Progressive Systemic Sclerosis (Scleroderma)

Skeletal findings in progressive systemic sclerosis (scleroderma)Daniel E. Furst2 Phillip J. Clements2
Richard H. Gold1
Received May 6, 1980; accepted after revision February2, 1981.
This work was supported in part by grants from the U.S. PublIc Health Service (RR—865),RGK Foundation, and Palos Verdes Junior Women's Club Scleroderma Fund.
I Department of Radiological Sciences, Center
for the Health Sciences, University of California, Los Angeles, CA 90024. Address reprint requests to L. W. Basett.
2Department of Medicine, UCLA School of Medicine, Los Angeles, CA 90024.
AJR 136:1121-1126,June1981 0361 -8o3x/81 /1 366-1 121 $00.00 ©American Roentgen Ray Society
Radlographs were reviewed of the chest, hands, and feet of 55 patIents with progressive systemic sclerosis. These patients had been selected so as to exclude overlap syndromes,particularly mixed connectivetissue dIsease.Soft tissue changes Included flexion deformIties, generalized or localized atrophy, and dyetrophlc calcifi catlons. While resorption of distal phalanges was the most common bony change, osteolysis In other sites (feet, ribs, and mandibles) was also frequent. Twelve of 55 patients showed radiographic evidence of inflammatory arthritIs, ranging from Isolated to generalizedjoint destruction,that could not be attributed to overlapwith rheumatoid arthritis or mixed connective tissue disease.
The musculoskeletal findings in progressive systemic sclerosis (P88) have been the subject of continued controversy, partly because most reports describe mixed populations of P55, mixed connective tissue disease (MCTD) [1], overlap syndromes of several connective tissue diseases and CREST patients (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangecta sia ) [2]. The 55 patients in this report had pure P55 as defined by the American Rheumatism Association [3], and were specifically screened to eliminate patients with the above syndromes.
MaterialsandMethods
Results
Soft Tissues
Flexion contractures of the hands were the most frequent radiographic finding (45 of 55 cases). Soft tissue atrophy over the distal phalanges was also common (43 of 55). Soft tissue atrophy was defined as a decrease in the ratio of soft
Skeletal Findings in Progressive Systemic Sclerosis (Scleroderma)
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Fig. 1 —¿A,Calcifications within soft tissues of fingers are usually on volar side.In thiscase,anextensortendonIs also calcified (arrowheads). B, CalcIfi catlons in Intervertebral discs (arrows).
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tissue to phalangeal base to under 20%, as suggested by Yune et aI. [5]. Calcium deposition in the hands was ob served in 14 of 55 cases. These were most often present in the digits although calcifications were also identified in tendons (one case) (fig. 1A), joints (two), triangular cartilage (one), paraspinous locations (two), and vertebral discs (one) (fig. 1B).
Localized Bony Resorption
The most common site of bony resorption was the tufts of the terminal phalanges in the hands (28 of 55) (fig. 2A). While the overlying soft tissues of the fingertips were usually atrophied, underlying bone loss was often greater than the soft tissue change. Occasionally the fingertips were corn
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PROGRESSIVE SYSTEMIC SCLEROSIS 1123AJR:136,June1981
Fig. 3.—A, Smooth extrinsic defects in distal radius (arrow) and ulna. B, Re sorption of upper parts of posterior as pectof ribs2—5(arrows).
Fig. 4.—A, In P55, cortIcal margin of mandiblemayberesorbed(arrows)with considerablebone destructIon.Peri odontal ligament space is widened around one of the premolars. (Courtesy of Stuart White, UCLA School of Den tistry.) B, In comparison, normal mandib ular angles are characterized by sharp cortical margins.
A B -j
pletely destroyed (fig. 2B) (acroscleroderma). A transverse band of resorption in the midshaft of the distal phalanx was observed in two patients (fig. 2C). Although bone resorption in the fingertips was progressive in most cases, there was evidence of improvement in three cases.
Circumscribed resorption of bone along the distal radius and ulna was seen in one case (fig. 3A). The two patients with resorption of the distal phalanges of the feet invariably had more advanced changes in the hands.
Seven of 55 patients exhibited destruction of the pos terosupenior one-third to one-half of ribs 2—6(fig. 3B). Six of these seven had no other evident sites of bone resorption.
Distal clavicular resorption was noted on the chest radio graphs of only one patient, but, because special acromio clavicular films were not obtained on any patients, this may not be representative of the true incidence of this finding.
Osseous resorption around the mandibular angles was
present in six of the 35 patients who had Panorex mandib ulan tomograms (fig. 4). All of these patients also had ob vious tuft resorption and two had rib resorption as well. The mandibular angle lesions were either infeniorly or posteriorly located. In three patients sequential radiographs revealed progression of the mandibular erosion. In two patients com plete or partial resorption of the coronoid processes was also present, and coronoid resorption was an isolated find ing in another patient.
Arthropathy
Joint erosions were found in the hand radiographs of 12 of 55 patients. Erosions were often similar to those seen in rheumatoid arthritis (fig. 5A): predominantly volar erosions of the metacarpophalangeal, proximal interphalangeal, and radiocarpal joints, beginning at the joint margins and, in
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Fi;@ —¿A.Dis@.@Iiti@ ird@ un ntr,@ctur@s @r@ct(rlstI(@@ f PSS In .iddit@ori. there ari rn@irginal rc siori@@ j@h@C@J@ s@ c@r@@1@ tr@;1t,@ ‘¿tensive Erosions of r.@dI()uIr@ir joint . tnd radiocarial narrowing
B ml C Erc@ion@on ior@,uI @rti:uIir @,ur@tE(irr:;v.s)@ fuiirid iii fiv@p@itientsvuith PSS
Fig. 6.—A, Destruction of almost all joints with extensive intraarticular calci fication was observed bIlaterally In hands.B.Erosivearthropathyinfirstand fifth metatarsophalangeal joints (ar rows).
BASSETT ET AL.
PROGRESSIVE SYSTEMIC SCLEROSIS 1125AJR:136, June 1981
A B Fig. 8.—A, Distal interphalangeal joints show severe destruction resembling ‘¿â€˜¿arthritismutilans. “¿B,
Same patient. Similar changes at first carpometacarpal joint. Fig. 7.—Distal or terminal phalangeal scle
rosis in five of 55 patients.
some cases, ultimately progressing to involve the entire articular surface. In six cases the ulnar styloid process was
eroded. In five cases erosions were found on the dorsal aspect of the hand. These were sometimes associated with volar erosions as well, but in two cases only dorsal erosions were present (figs. SB and SC). In another patient severe joint destruction was associated with deposition of calcium within the joints (fig. 6A).
There was radiographic evidence of erosive arthritis in the feet of four of 12 patients with joint disease in the hands (fig. 6B), but an instance of foot involvement without disease in the hands was not encountered.
Terminal Phalangeal Sclerosis
Sclerosis of the distal half of the terminal phalanx was present in five of 55 patients (fig. 7).
Discussion
Flexion deformities and soft tissue atrophy are the most frequent observations on hand radiographs of P55 patients, and directly relate to the development of taut, ‘¿â€˜¿hidebound― skin in the hands.
The calcifications in PSS are dystrophic in origin, the calcium deposits reflecting a response to tissue damage. Thus they are most commonly seen in the fingers, especially the fingertips. However, calcifications may occur in any location, even within joints in the presence or absence of joint destruction [6].
Bone resorption in PSS is most frequently seen in the distal phalangeal tufts. In this site it is almost always pro gressive although in our series there was sequential radio graphic improvement in three patients. The radiographic improvement did not correlate with any parameter of clinical improvement. Transverse bands of resorption in the mid shaft of the distal phalanges is an unusual and interesting finding that is also found in association with exposure to the products of polymerization of vinyl chloride [7], as well as in association with cranioskeletal dysplasia [8]. Bony resorp tion in the toes was fan less common than in the fingers and
was always less severe. The cause of superior marginal rib resorption in PSS is uncertain [9, 10].
Mandibular angle erosions were present in six of 35 patients examined with panoramic mandibular tomography. Oral radiographic findings in P55 were reported by Stafne and Austin [1 1] in 1944. They found an increased width of the periodontal membrane in 7% of patients. Resorption of the posteroinferior cortex of the angle of the mandible was first described in association with P55 in 1959 [1 2]. Man dibular resportion in P55 may be associated with a re stricted oral aperatune[13] and pathologic fracture [14]. The cause of mandibular resorption is unknown but pressure atrophy resulting from skin thickening or osseous ischemia may be responsible [15]. Terminal phalangeal sclerosis is not understood, but is a well documented finding in other collagen vascular diseases, particularly rheumatoid arthritis.
Polyarthralgia or arthritis occurs within 1 year of disease onset, or is the initial symptom, in 40% of P55 patients [1 6]. Radiographic evidence of arthropathy was present in
the radiographs of 12 of 55 patients. The findings varied from a solitary erosion of the ulnar styloid to severe destruc tion of multiple joints of the hands and feet. In one case this was accompanied by calcium deposition within all involved joints. These radiographic changes developed during the course of interval examinations in seven of these 12 pa tients. It is worth emphasizing that the rheumatoid factor was negative in 10 of the 12 patients and was not positive in a higher proportion of the patients with arthropathy than
in those without clinical or radiographic evidence of joint abnormalities. Further, there was no clinical evidence for rheumatoid arthritis in any of the patients.
The disparity between the paucity of latex agglutination reactions and the frequency of articular lesions is consistent with the findings of other investigators [17]. When erosions were present they were often bilaterally asymmetric and were sometimes located dorsally—features not character istic of rheumatoid arthritis. The reason for dorsal erosions is obscure, but they may result from the pressure of adjacent extensor tendons rubbing against the bones in contracted atrophic hands. A grating sensation or friction rub over tendinous areas, including the fingers, is a well documented phenomenon in patients with P55 [18]. Selective involve
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1126 BASSETT ET AL. AJR:136, June 1981
ment of the first metacarpophalangeal joint has been re ported in P55 [19]. This joint was involved in four of our 12 patients with radiographic arthropathy. An unusual arthritic pattern in P55 recently reported by Brower et al. [20] was not observed in our original series of 55 patients. Subse quently it was encountered in the proximal and distal inter phalangeal joints of another patient with P55 at our institu tion, and consisted of severe destruction of articular sun faces with increased space between the remaining smoothly marginated subchondral bony surfaces; this pattern resem bled the end-stage of erosive polyarthnitis—so called “¿an thnitis mutilans,' ‘¿or the ‘¿â€˜¿pencil-in-cup'‘¿appearance of neu ropathic joint disease (fig. 8). This case, along with 12 of 55 patients in our study group, emphasize the presence of a nonrheumatoid erosive arthropathy in progressive systemic sclerosis.
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