No está claro cuán grande es el papel de los antibióticos https://antibioticos-wiki.es en las relaciones competitivas entre los microorganismos en condiciones naturales. Zelman Waxman creía que este papel era mínimo, los antibióticos no se forman sino en culturas limpias en entornos ricos. Posteriormente, sin embargo, se descubrió que en muchos productos, la actividad de síntesis de antibióticos aumenta en presencia de otros tipos o productos específicos de su metabolismo.
Untitled
Therapeutic Advances in Musculoskeletal Disease
Sternocostoclavicular hyperostosis: a review
! The Author(s), 2011. Reprints and permissions:http://www.sagepub.co.uk/
Abstract: Sternocostoclavicular hyperostosis (SCCH) is a chronic inflammatory disorder which
presents with erythema, swelling, and pain of the sternoclavicular joint. Approximately one halfof patients have acne or pustular lesions with the best described association being withpalmoplantar pustulosis (PPP). Extrasternal articular disease occurs in about a quarter ofpatients. The inflammatory process spans several years and has periods of exacerbationfollowed by remission. The histologic picture demonstrates a sterile osteomyelitis of thesternum and medial end of the clavicle. The diagnosis of SCCH is confirmed radiographicallyby hyperostosis and sclerosis of the sternum with involvement of the first rib on computedtomography (CT). The focal uptake of radiopharmaceutical on bone scintigraphy called the‘bullhead’ sign is highly sensitive of SCCH. Treatment is aimed at easing pain and modifying theinflammatory process. Evidence over the last two decades suggests a role for intravenousbisphosphonates and tumor necrosis factor alpha inhibitors. A low level of awareness of SCCHoften leads to a delay in diagnosis. This translates into significant morbidity and brings apsychological burden. Untreated chronic inflammation of the sternoclavicular joint leads torestricted mobility and secondary degenerative joint changes. In the search for a diagnosis,patients often undergo multiple serologic and imaging studies and in the experience of theauthor are referred to multiple specialists before a correct diagnosis is made. Greaterawareness of SCCH is needed to prevent the irreversible physical and psychological impair-ments associated with the disease.
Keywords: sternocostoclavicular hyperostosis, anterior chest wall, SAPHO syndrome,sternoclavicular joint
literature in 1975 [van der Kloot et al. 2010;
Although it was speculated that the clinical and
Ko¨hler et al. 1975]. In 1979 Sonozaki and
radiographic manifestations of sternocostoclavi-
described in the early 20th century [Chigira and
based on the sternoclavicular findings in a
Shimizu, 1989] the first case of SCCH appeared
cohort of over 20 Japanese patients. By the
in the Japanese medical literature in 1967 when
early 1990s SCCH had over 50 synonyms such
Sasaki reported a Japanese patient who had
as pustulotic arthro-osteitis, pustulotic arthro-
‘bilateral clavicular osteomyelitis accompanied
osteopathy, hyperostotic pustulopsoriatic spon-
by palmar and plantar pustulosis’ [Dihlmann
and Dihlmann, 1991, p. 250; Sasaki, 1967]. In
syndrome [Dihlmann and Dihlmann, 1991]. A
1974 Sonozaki and colleagues reported the first
more commonly applied term has been the acro-
case of SCCH in English [van der Kloot et al.
nym SAPHO (synovitis, acne, pustulosis, hyper-
2010; Sonozaki et al. 1974]. Cases published
ostosis, and osteitis) syndrome [Chamot et al.
between the late 1960s and early 1970s reported
1987]. It was first introduced by Chamot and
patients with bilateral hyperostosis of the clavi-
colleagues in 1987 to describe a group of bone
cles uniformly associated with pustulosis of the
and joint abnormalities associated with skin
palms and soles [Fritz et al. 1992]. The first
lesions. While there is overlap between the clini-
case of SCCH reported in the Western medical
cal, histological, and radiographic manifestations
literature with similar clinical and radiographic
of SAPHO syndrome and SCCH, it is recognized
Therapeutic Advances in Musculoskeletal Disease 3 (2)
disorder [Schilling, 2003] and SCCH continues
anteriorly and posteriorly by ligaments as well as
to appear as a distinct medical and diagnostic
superiorly by the interclavicular ligament. The
term publishable in the literature [van der Kloot
costoclavicular ligaments extend from the carti-
et al. 2010]. For these reasons SCCH will be the
lage of the first ribs to the clavicles and are each
term used to categorize the clinical, histologic,
separated into an anterior and posterior sheet by
and radiographic findings discussed in this
Surrounding structures like the sternomanubrialjoint form a symphysis rather than a true syno-
SCCH is considered a rare disorder [van der
vium and the costochondral joints form synchon-
Kloot et al. 2010] with no well defined incidence
because the current literature consists of case
sternoclavicular joint area possesses a variety of
reports and case series. It is a disorder of midlife
structures that can potentially be involved in an
with a relatively broad age range. A recent series
inflammatory arthritis or enthesitis [Katz et al.
of 52 patients reported ages ranging between
24 and 79 years [van der Kloot et al. 2010]. One explanation for such a broad age range is
SCCH typically starts with nonspecific inflam-
that the clavicle can be involved in other arthritic
mation of the sternoclavicular ligaments [Fritz
disorders. Condensing osteitis of the clavicle, a
et al. 1992]. Surrounding soft tissues may also
rare but benign condition which has clinical and
be involved in the early inflammatory process
radiographic findings similar to that of SCCH,
[Saghafi et al. 1993] and perichondritis appears
can occur in women in their late childbearing
to play a more important role than a true arthritis
years without a dermatosis [Noble, 2003; Tait
[Chigira and Shimizu, 1989] but eventually an
et al. 1994]. Chronic recurrent multifocal osteo-
erosive arthritis develops [Fritz et al. 1992;
myelitis (CRMO), a disorder which has the same
Dihlmann and Dihlmann, 1991]. As the inflam-
hyperostotic changes in the sternum and clavi-
matory process continues, endosteal and perios-
cles, occurs exclusively in patients from infancy
teal bone formation occurs along with the growth
to young adulthood [Fritz et al. 1992]. While
of partially destructive fibrous tissue [Fritz et al.
some thought that SCCH was on a continuum
1992]. Chronic inflammation leads to progres-
with CRMO [Fritz et al. 1992; Dihlmann and
sive hyperostosis and eventual soft tissue ossifica-
Dihlmann, 1991], more recent authors have
tion [Fritz et al. 1992; Sartoris et al. 1986]. After
argued that the two diseases have no distinguish-
several years of chronic inflammation, fusion of
able features [Katz et al. 1989]. Insight about the
the sternoclavicular joints may occur [Fritz et al.
ethnic prevalence of SCCH information remains
1992]. Concomitant with the hyperostosis involv-
limited because case series variably report data
ing the sternoclavicular joint, similar entheso-
about the ethnicity of their patients. While the
pathic and hyperostotic changes may involve the
majority of patients may be Japanese, European/
shafts of the long bones, sacroiliac joints, or
White patients make up a significant minority
peripheral joints [Johnston and Elston, 1998].
[Johnston and Elston, 1998]. To date, only oneother case has reported a patient of African
Histologically SCCH resembles a nonspecific
descent [Kalke et al. 2001]. While a male
inflammatory cellular response akin to that seen
predominance was previously reported, recent
[Nungu et al. 1997; Economou et al. 1993;
[van der Kloot et al. 2010; Economou et al.
Sartoris et al. 1986]. The disorder starts with an
ossifying periostitis, with newly formed periostealbone fusing with the existing cortex and the orig-
inal trabecular bone adapting to this alteration
The sternoclavicular joint is a true synovial lined
[Kalke et al. 2001; Ko¨hler et al. 1977]. As the
articulation composed of the inferior portion of
inflammatory response continues, bone turnover
the medial head of the clavicle, an indentation in
accelerates, resulting in formation of woven bone
the upper outer portion of the sternum, and the
and the appearance of osteoid seams [Kohler
cartilage of the first rib [Yood and Goldenberg,
et al. 1977]. Thickening of trabecular bone,
1980]. The joint is divided into two compart-
formation of osteoid and fibrous tissue, and
ments which in nearly all people is separated by
an intra-articular disc. The articular capsule sur-
[Fritz et al. 1992; Sartoris et al. 1986]. In some
rounding the sternoclavicular joint is reinforced
angiogenesis occurs [Fritz et al. 1992; Sartoris
a ‘squared shoulder’ position [Economou et al.
et al. 1986]. Inflammation at junctional sites
may slowly extend to involve all of the enthesesaround the manubrium [Economou et al. 1993].
Extrasternal articular manifestations may be
Eventually the inflammatory process diminishes
encountered in SCCH. There is limited informa-
or resolves leaving the characteristic hyperostotic
tion on whether or not these manifestations
bone changes in the sternoclavicular area and a
precede, occur during, or develop after the
nonspecific osteosclerosis [Fritz et al. 1992;
onset of sternoclavicular area inflammation.
Multiple reports since the early 1980s suggestthat axial skeleton manifestations can occur in
To date no etiology has been linked to the path-
ogenesis of SCCH. Based on the response of
commented on radiographic changes only [Fritz
early case reports to antibiotics an infectious trig-
et al. 1992; Sartoris et al. 1986]. Information
ger was initially an appealing etiology, however
about the symptoms associated with axial skele-
more recent case series have cast significant
ton inflammation remains limited. Symptomatic
peripheral arthritis may occur in up to 22% of
Freyschmidt and Sternberg, 1998; Taylor and
patients [Kalke et al. 2001; Fritz et al. 1992].
Dawes, 1992]. The association of SCCH with
The peripheral arthritis of SCCH is typically an
oligoarthritis [Fritz et al. 1992] that preferentially
conclude that both conditions were triggered by
involves large joints [Kalke et al. 2001] with the
a ‘bacterid reaction’ [Chigira and Shimizu,
1989]. Propionibacterium acnes was implicated as
followed by the wrist and less commonly the
a possible infectious agent in patients with SCCH
proximal interphalangeal joints [Kalke et al.
and palmoplantar pustulosis (PPP) in the 1980s,
but little has been published since then tosupport such an association [Saghafi et al. 1993;
Since it was first recognized, SCCH has classi-
changes, specifically PPP. In addition to PPP,
other dermatoses associated with SCCH are pus-
SCCH very infrequently may be asymptomatic
tular psoriasis, acne conglobata, acne fulminans,
with radiographic findings only [Katz et al.
hidradenitis suppurativa, and psoriasis vulgaris
1989] but in most cases the disorder presents
with pain and palpable tenderness in the sterno-
Dihlmann, 1991]. A recent study reported that
clavicular region [Dihlmann and Dihlmann,
53.9% of patients had current or prior skin
1991; Chigira and Shimizu, 1989]. Local swell-
manifestations [van der Kloot et al. 2010].
ing and warmth is almost always present [Chigira
In the majority of patients (70%) the interval
and Shimizu, 1989]. Symptoms can be unilateral
between the onset of skeletal symptoms and
skin disease was less than 2 years [Freyschmidt
develop bilateral involvement [Prevo et al. 1989;
and Sternberg, 1998] yet patients may present
Sartoris et al. 1986]. Symptoms are often chronic
with PPP 2 years before SCCH or up to 5 years
in nature and have a relapsing and remitting
later [Nungu et al. 1997; Sartoris et al. 1986].
course [Kalke et al. 2001; Economou et al. 1993; Prevo et al. 1989; Sartoris et al. 1986;
There are no laboratory tests that are diagnostic
Ko¨hler et al. 1977]. Pain is typically reported in
of SCCH and the value of serologic testing is to
the shoulder, neck, or upper anterior chest wall
exclude alternative disorders [Nungu et al. 1997].
area with swelling and tenderness of the sterno-
While initial case reports documented markedly
clavicular area invariably present [Kalke et al.
elevated values for inflammatory markers such
2001; Economou et al. 1993]. Resultant to
inflammation of the medial clavicle, motion of
a2-globulin, and C-reactive protein, it is now
recognized that changes in these labs do not
[Economou et al. 1993; Chigira and Shimizu,
relate to the disease activity or course [Nungu
et al. 1997]. Although SCCH is a hyperostotic
provoked by shrugging or retracting the ipsilat-
process, only normal to slight elevations in the
eral shoulder [Kalke et al. 2001]. In advanced
cases, the shoulder girdle may become fixed in
[Nungu et al. 1997; Fritz et al. 1992]. Tests for
Therapeutic Advances in Musculoskeletal Disease 3 (2)
rheumatoid factor and other immunological tests
multiple ribs and ‘E’ for concomitant extrasternal
are usually negative [Economou et al. 1993].
manifestations [Fritz et al. 1992]. These modifi-
Although patients of European descent may
cations have been included in Table 1.
demonstrate a slightly increased prevalence ofHLA-B27 positivity, no clear relationship to
Sternal involvement in SCCH can involve the
first to fourth ribs [Prevo et al. 1989; Sartoris
et al. 1986; Ko¨hler et al. 1977] but some caseseries have reported involvement to the seventh
rib [Fritz et al. 1992]. Ossification involving or
beyond the eighth rib has not been reported
SCCH are ossification progressing to hyperosto-
[Fritz et al. 1992]. Radiographic axial involve-
sis of the clavicles, sternum, and anterior upper
ment may appear as erosive changes and exuber-
ribs [Prevo et al. 1989; Dehdashti and Siegel,
ant bone formation along the anterior aspects of
1989]. Early in the disorder it is possible that
the vertebral bodies [Karagevrekis et al. 1990;
no radiographic changes may be present, thus
Dehdashti and Siegel, 1989]. It can also appear
the prominence of ossification and hyperostosis
as sclerotic changes without proliferative or
depends on disease chronicity. Recognizing this,
Sonozaki and colleagues in the late 1970s
Anywhere from 41% to 55% of patients in vari-
proposed a radiographic staging system for
ous case series have had radiographic findings
SCCH [Dehdashti and Siegel, 1989]. This is
[Fritz et al. 1992; Sartoris et al. 1986]. Any part
of the cervical, thoracic, or lumbar spine may be
graphic stage at the time of diagnosis is stage II
involved, and the changes reported have been
[Fritz et al. 1992; Dehdashti and Siegel, 1989]
compared to an ‘erosive spondyloarthropathy’
and multiple authors have reported that such
changes infrequently progress even in the pres-
Radiographic changes similar to those seen in dif-
fuse idiopathic skeletal hyperostosis and ankylos-
et al. 1993; Fritz et al. 1992; Prevo et al. 1989;
Sartoris et al. 1986]. Chigira and Shimizu added
a stage 0 to the Sonozaki staging in which noossification
As a more sensitive imaging modality, computed
[Chigira and Shimizu, 1989]. Joint spaces in
tomography (CT) has the ability to detect abnor-
the sternoclavicular region initially are preserved,
malities in the sternoclavicular area not obvious
but as the disorder progresses radiographic
on radiographs (stage 0). CT facilitates earlier
evidence of erosive changes predominantly in
detection of pathologic changes in SCCH and
the sternoclavicular joint may be detected [Fritz
changes found can differ from those on radio-
et al. 1992; Dihlmann and Dihlmann, 1991].
graphs [Economou et al. 1993]. Chigira and
Recognizing that ossification and hyperostotic
Shimizu 1989 devised a staging system for the
CT findings for SCCH [Economou et al. 1993;
region, Fritz and colleagues further modified
the Sonozaki staging in 1992 by adding the qual-
shown in Table 2. Typical CT changes involving
ifiers of ‘R’ for concomitant involvement of
the sternoclavicular joint are shown in Figure 1.
Table 1. Radiographic staging of sternocostoclavicular hyperostosis proposed by Sonozaki et al. [Dehdashtiand Siegel, 1989; Sonazaki et al. 1979] with modifications by Chigira and colleagues [Chigira and Shimizu, 1989]and Fritz and colleagues [Fritz et al. 1992].
No ossification noted in the sternoclavicular region
Mild ossification confined to costoclavicular ligaments
Spread of ossification beyond the costoclavicular ligaments to surrounding
tissues with loss of definition of the inferior margin of the clavicle andsuperior margin of the first rib
Hyperostosis of the inferior and superior margins of the clavicles
Qualifiers: ‘R’, involvement of multiple ribs; ‘E’, extrasternal manifestations
Given the sensitivity of CT, it is possible to ‘over-
2001; Dihlmann et al. 1997]. The predominant
call’ age-related changes such as calcification and
uptake in these areas has lead several authors to
ossification of the costal cartilage [Dihlmann
label this configuration as the ‘bullhead’ sign
and Dihlmann, 1991, p. 261]. Dihlmann and
[Freyschmidt and Sternberg, 1998; Dihlmann
Dihlmann reported that ‘both inflammatory
et al. 1997]. This scintigraphic finding is shown
enthesopathy of the costoclavicular ligament
in Figure 2. This sign is defined as the ‘typical,
and the occurrence of small hyperostotic foci of
mostly symmetrical high uptake of the sternocla-
at least 5 mm diameter at the sternal end of the
vicular region like a ‘‘bullhead’’: the manubrium
first pair of ribs are among the diagnostically
representing the upper skull of the bull and the
decisive early findings’ in the sternoclavicular
horns corresponding to the inflamed sternoclavi-
1991]. When these changes are identifiable they
[Freyschmidt and Sternberg, 1998, p. 808].
can help establish SCCH as a diagnosis early in
Although the shape can be modified or slightly
asymmetric, the characteristic pattern is consid-ered to be always visible [Freyschmidt and
Scintigraphy is another imaging modality able to
Sternberg, 1998]. While some authors have
identify uptake in characteristic regions when
argued that the ‘bullhead’ sign is both sensitive
and specific for SCCH [Jurik, 1991], the specifi-
changes are absent or subtly abnormal [Kim
city of this finding is dependent on the presence
et al. 2001; Nungu et al. 1997; Dehdashti and
of skin lesions. When patients with pustular skin
Siegel, 1989; Sartoris et al. 1986]. Increased
changes are removed the specificity has been
uptake is almost always found in the sternoclavi-
found to be lower than that noted earlier
cular joint area, the costal cartilages of the first
[Dihlmann et al. 1997]. To distinguish patients
and second ribs, and the manubrium [Kalke et al.
who have true sternoclavicular joint involvement
Table 2. Computed tomographic (CT) staging of sternoclavicular hyperostosis proposed by Chigira and col-leagues [Chigira and Shimizu, 1989; Economou et al. 1993].
Hyperostosis of the cartilaginous surface of the first ribs with protrusion noted at the
sternocostal junctions. No definite thickening of the soft tissues around the cartilageand bones noted
Stage I findings with cartilaginous portion of the first rib becoming dense with irregular
Stage II findings with general hyperostosis of the clavicles, sternum, and first ribs. Marked
ossification of the costoclavicular ligaments present
Figure 1. Axial (left) and coronal (right) images of right sternal (black arrow) and clavicular (black arrowhead)erosions with early enthesophyte (white arrow) involving the right clavicular head.
Therapeutic Advances in Musculoskeletal Disease 3 (2)
cancer, lymphoma, or metastatic breast or pros-tate cancer should also be considered in anypatient at risk for these neoplasias [Economouet al. 1993; Dihlmann and Dihlmann, 1991;Katz et al. 1989; Prevo et al. 1989]. Asepticnecrosis of the clavicle (Friedrich’s disease) anda healing fracture are other conditions to considerearly in the disease course [Freyschmidt andSternberg,
Scintigraphically it is possible to distinguishthese two disorders from SCCH because theytypically do not cause bilateral disease and donot involve joints as well as adjacent bones[Freyschmidt and Sternberg, 1998]. As discussed
Figure 2. Bone scan image demonstrating increased
earlier, condensing osteitis of the clavicle is a
uptake in the area of the sternoclavicular joints
benign disorder thought to be a mechanical
appearing like the head of a bull, the ‘bullhead’ sign
phenomenon [Lissens et al. 1990; Sartoris et al.
1986; Yood and Goldenberg, 1980]. Changes arerestricted to the medial portion of the claviclewith
caused by SCCH, Dihlmann and colleagues rec-
[Dihlmann et al. 1997; Tait et al. 1994; Yood
ommended anterior and posterior scanning with
increased uptake in both areas being consistentwith an inflammatory process [Dihlmann et al.
Differentiating SCCH from the seronegative
spondyloarthropathies may be challenging atany stage of the illness since this group of arthri-
In addition to the somewhat distinctive ‘bull-
tides may involve the joints of the sternoclavicular
head’ sign, scintigraphy also has the advantage
area and distinguishing features may not appear
of identifying additional sternal and extrasternal
involvement, even when the patient is asymptom-
Histologically the mixture of destructive and
proliferative bone changes may suggest a seroneg-
Sternberg, 1998; Dihlmann et al. 1997; Fritz
et al. 1992]. In one series scintigraphic changes
Sternberg, 1998; Dehdashti and Siegel, 1989].
in the axial skeleton were noted in up to 67% of
To date, in patients with SCCH there have
patients and in the peripheral joints in 29%
been no reports of symptoms, examination find-
[Freyschmidt and Sternberg, 1998]. It has been
ings, or imaging abnormalities involving the gas-
argued that scintigraphy should be considered in
trointestinal tract, the genitourinary tract, or the
patients with anterior chest wall symptoms suspi-
eyes, even after long periods of follow up. Thus,
cious for SCCH since it can be useful investigat-
patients with involvement of these organ systems
ing the extent and intensity of disease [Dihlmann
are more likely to have a seronegative spondyloar-
thropathy or possibly the SAPHO syndrome andnot SCCH.
Differential diagnosisA variety of conditions can produce the clinical or
radiographic findings typical of SCCH. Early in
A variety of therapies have been utilized to miti-
the disease when symptoms present with or with-
gate the symptoms of SCCH as well as modify
disease progression. Traditional analgesic agents
such as nonsteroidal anti-inflammatory drugs
[Dehdashti and Siegel, 1989; Katz et al. 1989].
(NSAIDs) have been tried in most case series
Salmonellosis, brucellosis, tuberculosis, staphylo-
(up to 94% in one study) [Kalke et al. 2001].
coccal infection, and syphilis are a few organisms
While improvement in pain with NSAIDs has
that can cause infection of the sternoclavicular
frequently been reported, the degree and dura-
joint or a subacute osteomyelitis [Taylor and
tion of relief is variable [Freyschmidt and
Dawes, 1992; Dehdashti and Siegel, 1989].
Sternberg, 1998; Nungu et al. 1997; Economou
A pancoast tumor with local extension, thyroid
Prevo et al. 1989; Ko¨hler et al. 1977]. No signif-
have a similar inhibitory effect on heterotopic
icant serologic or radiographic improvements
bone growth. Case reports suggesting success
have been reported with the routine use of
with the use of pamidronate in patients with
NSAIDs. In the absence of information to sug-
refractory symptoms have led to the use of the
gest a long-term disease-modifying benefit from
newer, more potent aminobisphosphonate iban-
the daily use of NSAIDs it is prudent to limit use
dronate [Ringe et al. 2006]. Ringe and colleagues
to pain control [Freyschmidt and Sternberg,
reported that three patients treated with ibandro-
1998]. In the absence of a clear infectious etiol-
nate experienced rapid improvement in pain,
ogy, antibiotics have no role in the treatment of
showed radiographic and scintigraphic evidence
of improvement, a gradual decline in ESR, and
improvement in bone mineral density despitetheir condition failing to respond to other phar-
In 1990 injectable calcitonin was first reported to
macologic and nonpharmacologic interventions
be effective in the treatment of some manifesta-
[Ringe et al. 2006]. A potential concern with
tions of SCCH in Japanese patients [Misaki et al.
the use of bisphosphonates is that, upon cessa-
1991; Lissens et al. 1990]. Injectable eel calcito-
tion, florid mineralization in areas of suppressed
nin within 48 h improved pain and swelling and
produced a quantitative improvement in scinti-
graphic tracer uptake but did not improve inflam-matory
The role of surgery in SCCH has been restricted
improvement was noted in any facet of SCCH
to patients whose condition has failed to respond
to all other therapeutic interventions [Taylor and
calcitonin [Misaki et al. 1991]. Traditional
Dawes, 1992]. Surgical resection of the medial
disease-modifying antirheumatic drugs such as
clavicle or the sternoclavicular joint seems to
methotrexate and sulfasalazine have been used
provide variable improvement in pain, although
in some patients but with very limited informa-
some authors report no improvement with this
tion available about their efficacy [Kalke et al.
intervention [Nungu et al. 1997; Chigira and
2001]. Cyclosporine A has also been used in
some patients and improvement in both theskin and bone manifestations of SCCH has
Long-term follow up of patients with SCCH sug-
1998], but renal toxicity and hypertension limit
gests most have a good prognosis [Kalke et al.
2001; Economou et al. 1993], although the
currently exists in the medical literature about
the role or efficacy of tumor necrosis factor
burden [van der Kloot et al. 2010]. To date, the
longest published follow up of a patient with
author’s experience personally treating several
SCCH has been 31 years [Economou et al.
patients with SCCH, methotrexate and sulfasala-
1993; Fallet et al. 1983]. In SCCH, there seems
zine have been marginally effective in improving
to be a period of about a decade in which painful
clinical symptoms and modifying the disease
exacerbations may occur with transient elevations
course whereas TNF-a inhibitors such as adali-
in ESR and persistence of activity on scintigrams
mumab and etanercept seem to be more effec-
[Economou et al. 1993; Ko¨hler et al. 1977].
tive. Prospective trials are needed to validate the
Spontaneous remission may occur in patients
efficacy of this class of medications.
after the sixth decade of life [Nungu et al. 1997]. Although radiographic changes typically
Therapeutic interventions have also focused on
progress slowly, after about 12 years of disease
activity ossification of the costoclavicular liga-
1970s ‘anti-inflammatory radiation therapy’ was
used in four out of five patients whose condition
Dihlmann, 1991]. In a recent study the delay in
had previously failed to respond to NSAIDs and/
making the diagnosis was strongly associated with
or corticosteroids [Ko¨hler et al. 1977]. Although
patients feeling they were not being taken seri-
the long-term follow up was limited and toxicity
ously, a higher level of emotional distress, more
was a concern, the authors suggested that lasting
health-related impairments in daily functioning,
remission could be achieved with this treatment
and increased unemployment [van der Kloot
modality [Ko¨hler et al. 1977]. Bisphosphonates
Therapeutic Advances in Musculoskeletal Disease 3 (2)
A frequently encountered complication of SCCH
and Sternberg, 1998; Dihlmann et al. 1997;
is chronic ipsilateral shoulder pain with a resul-
Nungu et al. 1997; Fritz et al. 1992]. The
‘bullhead’ sign on scintigraphy appears to
inflammation and ultimately fusion of the sterno-
be a highly sensitive sign of SCCH [Kimet al. 2001]. The specificity of this sign is
clavicular joint. Since the 1970s it has been
recognized that upper extremity edema and
venous congestion from retrosternal soft tissue
1998]. Involvement of any rib on radiography
proliferation and secondary occlusion of the
or scintigraphy beyond the seventh should
subclavian vein can complicate longstanding
decrease suspicion for SCCH [Fritz et al.
SCCH [Economou et al. 1993; Fritz et al.
1992; Dehdashti and Siegel, 1989; Prevo et al. 1989; Sartoris et al. 1986; Ko¨hler et al. 1977].
The role of biopsy and histology helps exclude
The retrosternal proliferation of soft tissue and
alternative diagnoses. Not all cases reported
bone creates a thoracic inlet syndrome by
have had biopsies of areas of inflammation and
compressing local venous structures [Fritz et al.
the histologic picture will evolve with chronicity
1992]. Other unusual complications include
of the disorder. Several authors have commented
aortic calcification [Watts et al. 1993], clavicular
that when histology was nonspecific the diagnosis
stress fracture with delayed healing [Kuroda et al.
was never made from biopsy alone [Freyschmidt
2005], and chronic sterile discharging sinuses
features attempt to incorporate the most perti-nent findings of SCCH that make it a unique
clinical entity and distinguish it from other
Confusion from overlapping nomenclature, wide-
chronic inflammatory disorders involving the
spread lack of familiarity with the disorder, and
delays in characteristic radiographic changes havehindered formulation of uniform criteria to
classify SCCH. There is often a considerable
SCCH is a chronic inflammatory disorder with a
delay in establishing the diagnosis, with the
predilection for the sternoclavicular joints but it
time to diagnosis in one case series between
may potentially involve other axial and peripheral
9 months and 5 years [Economou et al. 1993].
joints and in some cases can be associated with
A recent study confirmed that diagnosis of
pustular skin changes as well. Nomenclature
SCCH was on average delayed for 5.6 years
discord has hindered the formulation of a stan-
[van der Kloot et al. 2010]. Incorporating obser-
dard definition of findings that constitute the
vations from other authors and multiple case
disorder. Rheumatologists as well as other clini-
series, the literature suggests that the criteria for
cians that evaluate patients with musculoskeletal
classifying SCCH should embrace two tenets
complaints should familiarize themselves with the
clinical and radiographic changes associated withSCCH because a delay in diagnosis has been
1. The presence of clinical inflammation in the
shown to be associated with irreversible long-
sternoclavicular area: pustulotic skin changes
term physical and psychological impairments.
are not always required for the diagnosis
Although treatment is currently limited, hope
[Karagevrekis et al. 1990]. Bilateral but occa-
may lie in the efficacy of newer bisphosphonates
sionally unilateral sternoclavicular involve-ment can occur. Axial skeleton or a large
joint oligoarthritis may be present. There isan
other organ systems such as the gastrointes-
The author confirms that this manuscript has
tinal tract, the genitourinary system, or
neither been submitted nor is simultaneously
being submitted elsewhere. No portion of the
data has been or will be published in proceedings
2. Radiologic, scintigraphic, or CT changes in
the sternoclavicular area imaging: scintigra-
or transactions of meetings or symposium vol-
phy has emerged as a useful tool to screen
umes. The views expressed in this article are
for inflammation of the sternoclavicular area
those of the author and do not reflect the official
as well as other typical but potentially asymp-
policy or position of the United States Air Force,
tomatic areas of involvement [Freyschmidt
Department of Defense, or the US Government.
Karagevrekis, C., Fallet, G.H. and Lagier, R.
The author has no financial support or other ben-
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Patient Name: Test, Patient Collection Date: 05/21/2007 Ordering Physician: Test, M.D. Received Date: 05/23/2007 Treating Physician: Training Report Date: 05/24/2007 Ordering Facility: Test Hospital US LABS Ref #: AP07-0000W Medical Record #: 000003 Date of Birth, Age: 01/27/1956, 51 Specimen ID #: 07-00001 Predictive/Prognostic Marker Analysis Specimen Site: L
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