Distal Madelung-Launois-Bensaude disease: an unusual differential diagnosis of acromelic arthritis.

Madelung-Launois-Bensaude (MLB) disease is uncommon and probably under-diagnosed. It is defined as a symmetrical proximal accumulation of fat. Its physiopathology is obscure, but favouring factors, like alcohol or corticosteroids, are well known. We describe, for the first time, an unusual case of distal form of the disease, involving hands and feet simultaneously. Moreover, this phenotype was associated with a proximal form of MLB, and new localizations in the elbows and knees have been identified. We review the literature concerning distal forms of MLB, and compare the features. This disease is an interesting new differential diagnosis of acromelic arthritis.

Increased exposure to statins in patients developing chronic muscle diseases: a 2-year retrospective study.

OBJECTIVE: Case reports have suggested that lipid-lowering drugs (LLDs), especially statins, could induce or reveal chronic muscle diseases. We conducted a study to evaluate the association between chronic muscle diseases and prior exposure to LLDs. METHOD: This was a retrospective study of chronic primary muscle disease cases newly diagnosed at the Toulouse University Hospitals between January 2003 and December 2004 among patients living in the Midi-Pyrénées area, France. All patients remained symptomatic for more than 1 year after drug withdrawal, or required drugs for inflammatory myopathy. Data on the patient's exposure to LLDs and to other drugs were compared with that of matched controls (5/1) selected through the Midi-Pyrénées Health Insurance System database. RESULTS: A total of 37 patients were included in the study. Of those, 21 (56.8%) suffered from dermatomyositis (DM) or polymyositis (PM), 12 (32.4%) from genetic myopathy, and 4 (10.8%) from an unclassified disease. The prevalence of exposure to statins was 40.5% in patients and 20% in controls (odds ratio (OR) 2.73, 95% confidence interval (CI) 1.21-6.14; p<0.01). There was a significant positive interaction between statins and proton pump inhibitors exposure (weighted OR 3.3, 95% CI 1.37-7.54; p = 0.02). Statin exposure rate was 47.6% among patients with DM/PM (OR 3.86, 95% CI 1.30-11.57; p<0.01). There was no difference between patients and controls for exposure to fibrates. CONCLUSION: Patients who developed chronic muscle diseases after the age of 50, including DM/PM, had a higher than expected frequency of prior exposure to statins. Further studies are needed to confirm this association and the role of proton pump inhibitors.

Comparison of the analgesic efficacy of pamidronate and synthetic human calcitonin in osteoporotic vertebral fractures: a double-blind controlled study.

Our aim was to compare the analgesic efficacy of pamidronate (PAM) and synthetic human calcitonin (CT) in intravenous infusion for recent painful benign vertebral compression in a randomised prospective double-blind study. Twenty-seven patients aged 49-85 years with painful benign non-traumatic vertebral compression were included in the study. They received either PAM (1 mg/kg) or synthetic human CT (1.5 mg) as an intravenous infusion. Pain and functional disability were evaluated before infusion, and 4 and 30 days afterwards. The pain score assessed on a visual analogue scale at day 0 was 5.94+/-2.47 in patients treated with PAM and 6.27+/-2.50 in patients treated with CT (p=0.74); at day 4, 4.8+/-2.80 with PAM vs 3.9+/-2.68 with CT (p=0.37); and at day 30, 3.6+/-3.13 with PAM vs 3.10+/-2.76 with CT (p=0.70). Spinal function scores were 18.21+/-3.17 at day 0 in patients treated with PAM vs 17.23+/-4.42 in patients treated with CT (p=0.69) and at day 30, 13.7+/-5.36 with PAM vs 12.33+/-3.22 with CT (p=0.68). We found no advantage of PAM over CT in a single intravenous infusion for the treatment of painful recent benign vertebral compression. Since CT is ten times less costly, its use should be preferred.

Safety of leflunomide plus infliximab combination therapy in rheumatoid arthritis.

OBJECTIVE: To analyse the safety of leflunomide plus infliximab combination therapy, in adult rheumatoid arthritis (RA) patients. PATIENTS: A retrospective study of 17 adult patients with active RA (DAS 28 = 5.94 +/- 0.88 at baseline) who were treated with a combination of leflunomide plus infliximab after failure of treatment with other DMARDs. 13 patients were treated for a minimum of 3 months with leflunomide without toxicity before beginning infliximab. Treatment was begun simultaneously with both drugs in 4 patients. Side effects (clinical and biological) and efficacy (DAS 28) were evaluated at each infliximab infusion (3 mg/kg at week 0, 2, 6 and then every 8 weeks). RESULTS: Thirteen patients experienced 20 types of side effects and 8 of them stopped the combination therapy. The causes of discontinuation were congestive heart failure (1 case), hypertension with thoracic pain (2 cases), eczematous skin patches (2 cases) and neutropenia (3 cases). No death was registered. Nine RA patients continuted the therapy with a median follow-up of 22 weeks. Only 4 of them experienced no side effects. Eight patients were positive for antinuclear antibodies (ANA) and 1 for double-stranded DNA (dsDNA) antibodies at study entry. After treatment, 13 and 5 patients tested positive respectively for ANAs and dsDNA antibodies. There was no relationship between discontinuation and ANA/dsDNA positivity. CONCLUSION: In this cohort, adverse events were not very different from those seen in patients on either treatment alone and the combination of leflunomide plus infliximab did not appear to be as badly tolerated as described in a previous study.

[Spontaneous pneumomediastinum in dermatomyositis: a pathophysiological approach using imaging]. / Pneumomédiastin spontané et dermatomyosite: approche physiopathologique par l'imagerie.

INTRODUCTION: Spontaneous pneumomediastinum is a rare complication of dermatomyositis. CASE REPORT: We report a case of pneumomediastinum with massive subcutaneous emphysema occurring in a female patient with dermatomyositis treated with cortico-steroids. CONCLUSIONS: Our case illustrates perfectly the mechanism of spread of air along the broncho-vascular structures and also explains the presence of pneumomediastinum in the absence of pneumothorax.

Does vascular endothelial growth factor in the rheumatoid synovium predict joint destruction? A clinical, radiological, and pathological study in 12 patients monitored for 10 years.

OBJECTIVE: Synovial angiogenesis is at the epicenter of rheumatoid pannus development and is largely dependent on vascular endothelial growth factor (VEGF). We sought to determine whether the VEGF level in rheumatoid synovial tissue is a marker for disease severity. PATIENTS AND METHODS: Twelve patients with rheumatoid arthritis (RA) underwent a clinical and radiological evaluation at the time of a synovial biopsy done during joint surgery required by RA progression (T1) and, on average, 10 years later (T2). Immunohistochemistry was used to detect and quantitate VEGF in the synovial biopsy taken at T1. RESULTS: VEGF labeling was seen on endothelial cells and macrophages in all 12 synovial biopsies. The amount of endothelial-cell VEGF labeling (assessed semi-quantitatively) was significantly correlated with Larsen score progression during the 10-year follow-up. The amounts of endothelial cell or macrophage VEGF labeling was not correlated with the joint count, radiological stage of the biopsied joint or progression of this stage, Larsen scores at T1 or T2, presence of rheumatoid factor, or presence of extra-articular manifestations. CONCLUSION: Our results suggest that the amount of VEGF in the rheumatoid synovium may be a marker for joint destruction in patients with RA.

Proposed classification criteria of psoriatic arthritis. A preliminary study in 260 patients.

UNLABELLED: Psoriatic arthritis probably owes to its radioclinical presentation its position as the most controversial and poorly understood of all major chronic inflammatory joint diseases. Differentiating psoriatic arthritis from ankylosing spondylitis and rheumatoid arthritis remains difficult. OBJECTIVE: To conduct a statistical analysis aimed at identifying clinical, radiological, and laboratory criteria for classifying psoriatic arthritis. PATIENTS AND METHODS: 260 patients were studied retrospectively, including 100 cases with psoriatic arthritis and 160 controls with ankylosing spondylitis meeting Amor's criteria (n = 80) or with rheumatoid arthritis meeting American College of Rheumatology criteria (n = 80). Mean disease duration was five years. Thirty-nine variables were recorded for each patient. Multiple logistic regression and discriminant analysis were used to select the classification criteria. RESULTS: Each of the two statistical methods selected the same nine criteria. After assigning a weighting coefficient to each of these criteria, sensitivity and specificity were better with the multiple logistic regression model (95% and 98%, respectively) than with the discriminant analysis model. CONCLUSION: Our classification criteria require further evaluation in multicenter prospective studies.

A back school program at the Toulouse-Purpan teaching hospital. Evaluation of 144 patients.

UNLABELLED: This article reports our experience with a five-day back school program focusing on active exercises, safe lifting, and occupational therapy. PATIENTS AND METHODS: The medical charts of 144 back school patients were reviewed retrospectively. Age, gender, type of low back pain, radiological findings, impact on work and psychological well-being, surgical history, and history of prior back school experience were recorded on the first and last back school session days (D1 and D5), after six months (M6), and after 12 months (M12). Efficacy was evaluated based on the number of days off work, Schöber's index, a visual analog scale pain score, and scores evaluating the active exercise, safe lifting, and occupational therapy techniques taught during the back school course. RESULTS: All evaluation scores were significantly better on D5 than on D1. This effect was sustained over time. The pain score fell by 50% between D1 and D5 (32.6 +/- 23.1 versus 16.3 +/- 18.5) and remained low at M6 and M12 (18.1 +/- 19.5 and 14.8 +/- 19.3) The number of days off work fell dramatically from baseline to M6 (51.2 +/- 63.8 vs 9.8 +/- 38.8) and remained low at M12. Forty-four patients were lost-to-follow-up between D5 and M6. CONCLUSION: Back school interventions are helpful in preventing recurrences of low back pain. Our promising data deserve to be confirmed in a larger study involving quality of life assessments, determination of the number of physician visits for low back pain during the M0-M6 and M6-M12 periods, and a comprehensive evaluation after 18 months.

A case of primary hypertrophic osteoarthropathy without skin involvement (Currarino's disease).

Hypertrophic osteoarthropathy is characterized by digital clubbing, arthropathy and periostosis of long tubular bones. Currarino's disease is an extremely rare variant of primary hypertrophic osteoarthropathy in which there is delayed closure of the fontanelles and an absence of skin involvement. Most reported cases have been in blacks. We report a case in a Caucasian adolescent.

Iliopsoas bursopathies. A review of twelve cases.

Synovial cysts are far less common at the hip than at the knee and usually occur in patients whose hip cavity communicates with the iliopsoas bursa. We report 12 cases of enlargement of the iliopsoas bursa, nine men and three women, with a mean age of 48 years. The six patients with septic bursitis had severe symptoms similar to those seen in septic arthritis of the hip. Chronic pain with or without a palpable inguinal swelling was the main symptom in the six remaining patients, some of whom had compression of neighboring structures making the diagnosis more difficult. Ultrasonography is the best first-line investigation in patients with an inguinal swelling. Computed arthrotomography with examination of the synovial fluid or magnetic resonance imaging should be performed as a confirmatory diagnostic test. Our series provides evidence of the efficacy of appropriate antimicrobial therapy in septic cases and of corticosteroid injections into the bursa or hip cavity in nonseptic cases.

Frozen shoulder: a new delayed complication of protease inhibitor therapy? .

We report three cases of frozen shoulder (including one with bilateral involvement) in human immunodeficiency virus (HIV)-positive patients under triple antiretroviral therapy. In each case, the diagnosis was confirmed by arthrography, and the classic causes of frozen shoulder were ruled out. We suggest that protease inhibitor therapy may have contributed to the development of frozen shoulder in these patients. Long-term follow-up of the increasing numbers of patients under triple antiretroviral therapy will confirm or refute this hypothesis.

Adhesive capsulitis of the shoulder: therapeutic contribution of subacromial bursography.

OBJECTIVE: To demonstrate the therapeutic value of subacromial bursography (with a steroid injection) in adhesive capsulitis of the shoulder inadequately improved by arthrographic glenohumeral distension with steroid injection. METHOD: Twenty cases of adhesive capsulitis documented by glenohumeral arthrography were studied prospectively. A steroid was injected during distension arthrography, which was followed by physical therapy. Subacromial bursography without steroid injection was done routinely for diagnostic purposes. Constant's simplified score and range of motion were determined in each patient at baseline and after one, three, six and 12 months. Patients who were inadequately improved after one to three months underwent repeat subacromial bursography with steroid injection, followed by physical therapy. RESULTS: Of the 20 patients, 13 were noticeably improved 1.7 months on average after the distension arthrography. Of the remaining seven patients, six were improved 0.7 months on average after the bursography with steroid injection. CONCLUSION: Glenohumeral distension arthrography with steroid injection followed by physical therapy is effective in expediting the spontaneously favorable outcome of adhesive capsulitis and also allows to confirm the diagnosis. However, the subacromial bursa is almost consistently involved. Subacromial bursography with steroid injection can be useful in cases that fail to respond to conventional therapy.

Synovial cyst at the C1-C2 junction and spondyloarthropathy.

A spinal synovial cyst is a rare extradural benign tumor generally located at the lumbar spine, arising at the facet joint capsule, and usually associated with degenerative changes. We describe a 64-year-old woman with a synovial cyst involving the quadrate ligament of the odontoid process, which caused neurologic signs. Her family history was positive for spondyloarthropathy. The radiologic investigations, preoperative differential diagnosis, and association of spondyloarthropathy with this rare benign foramen magnum tumor are of particular interest.

Fluoroquinolone induced tendinopathy: report of 6 cases.

We describe 6 cases of fluoroquinolone induced Achilles tendinitis in 4 women and 2 men, mean age 68.6 years. Patients presented with pain and swelling of sudden onset, which was most often bilateral. Tendon rupture was frequent, accompanied by nodules and ecchymoses. The diagnosis was clinical, occasionally ultrasonography was helpful; the role of magnetic resonance imaging has yet to be defined. Certain risk factors were found, particularly association with longterm steroid therapy, and close surveillance of high risk subjects is mandatory. Although proper dosage and duration of treatment were respected, the principal fluoroquinolones were clearly incriminated. We found no correlation between treatment duration and the degree of involvement. Nevertheless, immediate discontinuation of the antibiotic and placement of both Achilles tendons at rest is essential. Early and appropriate management did not prevent prolonged recovery times and there was always a risk of functional sequelae. This side effect is class related and rare. Its physiopathologic mechanism is poorly understood.