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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.
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