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Frequency of skeletal disease, arthro-osteitis, in patients with pustulosis palmoplantaris.

Fourteen randomly selected patients with pustulosis palmoplantaris were examined to determine the frequency of skeletal involvement. Symptoms and both clinical and radiographic signs of skeletal disease occurred in five patients (36%). Four patients had erosive and/or sclerotic changes in the region of the sternoclavicular, the first sternocostal, and/or the manubriosternal joint, in two with additional ossification of the costoclavicular ligament. Two patients had spinal involvement, one paravertebral ossifications, and the other sequelae of anterior spondylodiskitis. Peripheral joint complaints without radiographically detectable erosions, but with periarticular new bone formation, occurred in three patients. The skeletal changes are probably not incidental and seems to constitute a seronegative spondyloarthropathy associated with pustulosis palmoplantaris.

Adult↗

Evaluation of joints using Tc 99m-MDP bone scintigraphy in patients with familial Mediterranean fever: should bone scans be used for diagnosis and follow-up?

Familial Mediterranean fever (FMF) is an autosomal recessively-inherited disorder typically manifested by recurrent attacks of fever and polyserositis. The articular disease occurs in 50-70% of patients. Bone scintigraphy is more sensitive in the diagnosis of arthritis than clinical examination or conventional radiological imaging, allowing earlier diagnosis through the visualization of disease in multiple sites. To assess joint involvements in FMF patients with or without joint symptoms, bone scintigraphy was performed in 36 patients with FMF and in 25 controls. There was arthritis in 72% of patients. Of these, 65% knee, 42% ankle, 50% sacroiliac, 8% elbow, 8% wrist, 4% sternoclavicular and 4% hip involvements were found. The sacroiliac joints with sacroiliac index higher than 1.34 were diagnosed as sacroiliitis, which was higher than 2 SD of normal. FMF is frequently associated with joint disease such as knee and ankle arthritis and sacroiliitis. This high incidence of sacroiliitis in our study has not been previously reported. This difference could be explained by the different methodology used for the screening of the joints. Thus, we recommend that bone scintigraphy can be used in patients with FMF to determine the presence of arthritis, especially in sacroiliac joints, even asymptomatic.

Adolescent↗

Operative treatment of the rheumatoid shoulder.

Rheumatoid arthritis can affect almost any joint. Shoulder involvement typically occurs late in the disease process and usually after other joints have manifested arthritic change. Any of the four shoulder articulations can be involved: scapulothoracic, acromioclavicular, sternoclavicular, and glenohumeral. In addition to bony involvement, many of the soft tissues of the shoulder joint can be affected. Early operative treatment includes synovectomy with or without bursectomy, which is indicated prior to radiographic evidence of arthritis. Early synovectomy provides for a slowing of the progression of the disease process. Patients who have incapacitating pain with loss of range of motion can benefit from total shoulder replacement. Most patients experience pain relief and some restoration of motion. The restoration of normal range of motion is dependent on anatomic reconstruction of the glenohumeral joint. Factors that can affect the range of motion include rotator cuff tears and the general health status and motivation of the patient. Although there is a 30% to 80% incidence of radiographic lucencies with nonconstrained prostheses, their presence does not indicate the need for revision surgery. Occasionally, there is medialization of the glenohumeral joint with central bony losses of the glenoid. The surgeon should try to bone graft the defect and lateralize the components. If there is massive medialization of the glenoid that is not reconstructable, then a hemiarthroplasty is the procedure of choice.

Arthritis, Rheumatoid↗

[SAPHO syndrome. Scintigraphic and radiologic findings].

We present the case of a 39 year old woman with a several year long history of palmoplantar pustulosis and occasional pain in the anterior wall of the thorax, lumbar spine and both sacroiliac joints. As a consequence of low grade fever, severe sternoclavicular pain and discomfort and swelling in the first left rib arches, the patient was admitted to hospital. Based on the clinical, radiologic, scintigraphic and cutaneous findings, the SAPHO syndrome was diagnosed. Bone scintigraphy is an accurate and early diagnostic test for this syndrome which also provides the advantage of being able to obtain a whole body scan.

Acquired Hyperostosis Syndrome↗

Primary ankylosing spondylitis: patterns of disease in a Brazilian population of 147 patients.

OBJECTIVE: To analyze patterns of disease in a population of Brazilian patients with primary ankylosing spondylitis (AS). METHODS: Retrospective study (1988-98) analyzing 147 patients with a diagnosis of primary AS according to the modified New York criteria. Selected patients had complete clinical (initial symptom, axial and peripheral involvement, heel enthesitis, extraarticular manifestations) and radiological (sacroiliac, lumbar, thoracic, and cervical spine) investigations, and these data were compared with sex, race, age at onset, and HLA-B27. RESULTS: There was a predominance of men (84.4%), Caucasian race (75.5%), adult onset (> 16 years, 85%), and positive HLA-B27 (78.2%). Family history of AS was noted in 14.3% of the patients. Pure axial AS was observed in 37 patients (25.2%). The predominant initial symptoms were inflammatory low back pain (61.9%) and peripheral arthritis (22.4%). Thoracic and cervical spine involvement was noted in 70.1% of the patients; radiological findings included syndesmophytes in 46.9% and "bamboo spine" in 20.4% of patients. The extraaxial joints most frequently involved were: ankles (39.5%), hips (36.1%), knees (29.3%), shoulders (19%), and sternoclaviculars (14.3%); heel enthesitis was present in 22.4%. Acute anterior uveitis was noted in 14.3% of patients. Male sex was associated with involvement of thoracic spine (p = 0.002), cervical spine (p = 0.002), and hips (p = 0.042), whereas female sex was associated with sternoclavicular (p = 0.024) involvement. Caucasian race presented higher frequency of positive family history (p = 0.023); there was no statistical significance of clinical and radiological variables compared with African-Brazilians. Juvenile onset AS presented higher frequency of ankle (p = 0.012) and knee (p = 0.001) involvement, heel enthesitis (p = 0.001), and total hip replacement (p = 0.038), whereas adult onset was associated with thoracic (p = 0.026) and cervical spine (p = 0.026) involvement and positive family history (p = 0.044). Positive HLA-B27 was associated with ankle involvement (p = 0.007) and heel enthesitis (p = 0.013). CONCLUSION: In this population women showed a milder axial involvement, Caucasian race presented axial and peripheral involvement similar to African-Brazilians, juvenile onset AS was associated with articular involvement of the lower limbs, and positive HLA-B27 was associated with ankle involvement.

Adolescent↗

Perichondritis (Tietze's syndrome) at the xiphisternal joint: a mimic of severe disease.

Tietze's syndrome (peristernal chondritis or perichondritis) is a self-limited, painful inflammatory condition of the costochondral, sternoclavicular, manubriosternal or xiphisternal junctions. It is benign, and affects all ages, sexes and races equally. The principal manifestations are pain and, frequently, a mass most commonly at the costochondral junctions. In 1974, C.J. described the first cases to be reported at the xiphisternal junction. We describe here 24 cases at that site. These patients' presenting symptoms suggested cardiac, pulmonary, intraabdominal and other severe disease processes. Diagnosis was made on clinical grounds based on a high index of suspicion. Treatment comprised infiltration of local anesthetic to the afflicted joints with prompt, complete and prolonged relief in 21 (87.5%) of the cases, and complete temporary relief in three (12.5%). We suggest that a possible mechanism for the referral of pain is the relationship of the xiphisternal joint to a variety of structures including autonomic nerves that accompany the internal mammary arteries.

Adolescent↗

[Usefulness of bone imaging in diagnosis of sternocostoclavicular hyperostosis].

Sternocostoclavicular hyperostosis (SCCH) is a rare disease that is characterized by hyperostosis and soft tissue ossification between the clavicles and the anterior part of the upper ribs. There have been few published reports of 99mTc-MDP bone imaging findings in this disease. Thirty-three patients who were suspected to have SCCH were studied with 99mTc-MDP bone imaging. Bone images were abnormal in 31 (94%) of 33 patients. Chest radiographs were available in 30 patients, but negative in 14 (46%) of them. Bone imaging revealed also radionuclide accumulations in other bones such as the vertebrae, femur or sacroiliac joints in ten (14 sites) of 33 cases. Bone scintigraphy was useful in the diagnosis of sternoclavicular hyperostosis.

Adult↗

Treatment outcomes for temporomandibular joint reconstruction after Proplast-Teflon implant failure.

A retrospective study of 107 patients (male, n = 13; female, n = 94) with 163 joints previously treated with Proplast-Teflon (PT; Vitek, Inc, Houston, TX) implants was performed. The average time in situ for the PT was 59.8 months (range, 2 to 126 months). Average length of follow-up was 84.6 months (range, 59 to 126 months). Only 12% of joints showed no significant osseous changes radiographically. Forty-five patients (42%) continue to have in situ PT implants and 36% of them experience pain that requires medication; 25% have developed an anterior open bite and malocclusion; 9% have limited vertical opening; and 40% are asymptomatic. Temporomandibular joint (TMJ) reconstruction after PT implant failure was performed with five different autologous tissues or a total joint prosthesis. Autologous tissues used to reconstruct the TMJ and the rates of success were as follows: 1) 31% free temporalis fascia and muscle graft with and 13% without sagittal split ramus osteotomy, 2) 8% dermis, 3) 25% conchal cartilage, 4) 12% costochondral grafts, and 5) 21% sternoclavicular grafts. The success rate decreased in all autologous tissue groups as the number of TMJ surgeries performed before reconstruction increased. Ankylosis was the most common cause of failure. Results of TMJ reconstruction with a total joint prosthesis were as follows: 1) 88% functional and occlusal stability of total joint prosthesis; 2) level of pain reduction was rated as 46% good, 38% fair, and 16% poor; and 3) an average interincisal opening of 27 mm at 24 months or less, and 33 mm at 25 months and beyond.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

[Acute rheumatic polyarthritis in young men].

As many as 200 patients with acute rheumatic fever were examined. Impairment of the joints in the form of classic migrating polyarthritic was detected in 96% of the cases. The knee (in 80%), talocrural (in 72%), slightly rare radiocarpal (in 21%), and elbow joints were mostly damaged. In addition to the above-indicated damage to the joints, one could see slightly atypical injuries in the form of monoarthritis (in 4%), arthritis of small articulations of the feet and hands (in 6%), and of the sternoclavicular articulations (in 3.5%). According to the cytological, biochemical and immunological findings, synovial fluid obtained from 11 patients suffering from acute rheumatic fever did not differ, significantly from synovial fluid of patients with reactive arthritis.

Acute Disease↗

Osteoarticular complications of brucellosis: a study of 169 cases.

Of 452 patients with brucellosis, 169 (111 male and 58 female) had osteoarticular complications. Brucella melitensis was isolated from the blood in 7.7% of the cases. Fever, chills, arthralgia, backache, high levels of C-reactive protein, positive rheumatoid factor, and splenomegaly were more frequent in osteoarticular brucellosis than in nonosteoarticular disease. Arthritis occurred in the hip joint in 90 cases (53%), knees in 61 (36%), sacroiliacs in 33 (20%), ankles in 25 (15%), elbows in nine (5.3%), shoulders in eight (5%), wrists in six (3.5%), and sternoclavicular arthritis occurred in three cases (1.8%). Spondylitis occurred in 10 cases (6%), osteomyelitis in four (2.4%), and tendinitis or bursitis in two (1.2%). Treatment with tetracycline or trimethoprim-sulfamethoxazole (TMP-SMZ) alone (four to eight weeks) or in combination with streptomycin (two to four weeks) resulted in a relapse rate of 16.6%. No relapses occurred in seven patients treated with repeated four- to six-weeks courses of rifampin plus tetracycline or TMP-SMZ plus streptomycin.

Adolescent↗

The sternoclavicular syndrome: experience from a district general hospital and results of a national postal survey.

OBJECTIVE: To report our local experience of the sternoclavicular syndrome and sample the experience of other rheumatologists in the UK. METHODS: We studied case records of 23 patients referred to the Southend rheumatology clinic and data obtained from a postal questionnaire survey of British rheumatologists. RESULTS: We describe 58 cases (20 males and 38 females, mean age 47.2 yr). The disease was unilateral in 40 patients. Shoulder and/or arm pain (38 cases) with limitation of shoulder movements was an important presenting feature; other presenting features were anterior chest wall pain (14 cases) and neck pain (15 cases). Peripheral joint involvement was seen in 12 cases. Skin rash was reported in 12 cases (psoriasis, 6; acne, 2; none had pustulosis). No patients had symptoms or signs of sacroiliitis, and HLA-B27 was negative in 22 out of 23 patients. 99Technetium scintiscanning showed increased uptake in the sternoclavicular region in 31/34 patients (91.1%), but not in the sacroiliac areas. Plain radiographs were abnormal in 18 cases (sclerosis, 9; erosions, 2; soft tissue swelling, 2; bony expansion, 5). CT and/or MRI scans (available in 27 cases) showed erosions in 12 and osteitis in 18. Available histology showed a variable picture, including inflammation, bone erosion, sterile osteomyelitis and fibrosis. The majority of patients (45) were treated with non-steroidal anti-inflammatory drugs: 12 received steroids and 10 received disease-modifying anti-rheumatic drugs (methotrexate, 4; sulphasalazine, 6). Follow-up information was available for 38 patients, of whom 14 became asymptomatic and 24 had chronic disease with intermittent flares. CONCLUSION: Sternoclavicular disease is not uncommon in the UK. It can present with pain in the shoulder, neck or anterior chest wall, and may be underdiagnosed. Our results do not show a link with acne or pustulosis. Features of spondyloarthropathies, such as sacroiliitis and HLA-B27 positivity, were rare in this survey.

Adult↗

Modified anterior approach to the cervicothoracic junction.

STUDY DESIGN: This study reports the experience with four patients regarding a modified anterior approach to the cervicothoracic junction. OBJECTIVES: This technique was evaluated with respect to extent of exposure, ease of technique, and postoperative morbidity. SUMMARY OF BACKGROUND DATA: Previously reported anterior approaches to the cervicothoracic junction have described either full sternotomy resection of the left sternoclavicular junction or osteotomy of the clavicle. A simplified approach was chosen using a partial sternotomy, which has not been described previously for approaches to the spine. METHODS: Four patients with metastatic disease, in the region of the cervicothoracic junction, required decompression and stabilization for palliation of symptoms. An anterior approach was required for decompression. A standard cervical approach was combined with a partial median sternotomy and transverse osteotomy through the synostosis between the manubrium and body of the sternum. In three patients, the left innominate vein was divided. Decompression and anterior stabilization were followed by posterior stabilization at an interval of 4 to 7 days. RESULTS: This procedure was simple to perform, requiring little additional operative time for opening or closure. It provided excellent exposure from C3-T4. There was no associated morbidity related to the division of the manubrium or innominate vein. CONCLUSION: Partial sternotomy combined with a standard cervical incision provides excellent exposure to the cervicothoracic junction from C3-T4. It is technically simple to perform and avoids the risk of injury to subclavian vessels inherent in resection of the clavicle or sternoclavicular junction. There is no additional morbidity associated with this approach.

Cervical Vertebrae↗

Differences in shoulder muscle activity between patients with generalized joint laxity and normal controls.

The aim of the present study was to analyze shoulder muscle activity in patients with generalized joint laxity and shoulder instability and to compare it with muscle activity recorded in healthy subjects from an earlier study. Electromyographic (EMG) activity was recorded from eight shoulder muscles in six patients using surface and intramuscular fine-wire electrodes. Recordings were made from the subscapularis, supraspinatus, infraspinatus, pectoralis major (sternoclavicular part), the anterior, middle, and posterior parts of the deltoid, and the latissimus dorsi. The EMG signal was low-pass filtered, full-wave rectified, and time-average. Normalization of the EMG allowed interindividual and intraindividual comparisons. During abduction and flexion, muscle activity in the anterior and middle parts of the deltoid was significantly decreased in the patients, and during internal rotation activity in the subscapularis was increased. As in healthy subjects, patients showed simultaneous activity in both those muscles producing the movement and in the antagonistic muscles. The altered muscle activity observed in patients with generalized joint laxity provides (1) a basis for understanding the mechanism of their shoulder instability and (2) the rationale for a physical training program for these patients.

Adult↗