Primary prevention of glucocorticoid-induced osteoporosis with intravenous pamidronate and calcium: a prospective controlled 1-year study comparing a single infusion, an infusion given once every 3 months, and calcium alone.

The aim of this study was to compare the action of two regimens of intravenous (iv) pamidronate in the primary prevention of glucocorticoid-induced osteoporosis (GC-OP). The primary purpose of the study was to determine whether any differences in bone mineral density (BMD) appeared after 1 year. A secondary endpoint aimed at assessing the remodeling parameters in order to better understand the mechanisms of action of the various regimens. Thirty-two patients, who required first-time, long-term glucocorticoid therapy at a daily dose of at least 10 mg of prednisolone, were studied. Simultaneously with the initiation of their glucocorticoid treatment, patients also were randomly allocated to receive a single iv infusion of 90 mg of pamidronate at the start (group A); a first infusion of 90 mg of pamidronate followed, subsequently, by an iv infusion of 30 mg pamidronate every 3 months (group B); and a daily 800-mg elemental calcium supplement given as calcium carbonate (group C), which also was taken by patients in groups A and B. Patients were matched for starting glucocorticoid doses, sex, menopausal status, and hormonal replacement therapy. Lumbar spine and hip (total and subregions) BMDs were measured at the outset and repeated at 6-month intervals by dual-energy X-ray absorptiometry (DXA; Hologic QDR-2000). Bone turnover was assessed by measurement of total and bone-specific serum alkaline phosphatase activity (B-ALP), serum osteocalcin (OC), and serum C-telopeptide cross-links of type I collagen (CTX). After 1 year, the mean BMD changes for groups A, B, and C were, respectively, 1.7, 2.3, and -4.6% at the lumbar spine; 1.2, 1.2, and -3.1% at the femoral neck; 1.0, 2.6, and -2.2% for the total hip region. No difference was observed between pamidronate regimens but a highly significant difference was observed between both pamidronate regimens and the control group at the lumbar spine (p < 0.001), at the femoral neck (p < 0.01), and for the total hip (p < 0.05). A significant decrease of serum C-telopeptide was observed, after 3 months, in groups A and B (p = 0.029), but a sustained decrease of bone resorption over time was observed only in group B. As far as BMD evolution over 1 year was concerned, iv pamidronate, given either as a single infusion or once every 3 months, effectively achieved primary prevention of GC-OP.

Pustulotic arthro-osteitis in children and adults. A report of 13 cases.

Thirteen cases of pustulotic arthro-osteitis are reported: 3 children and 10 adults. The clinical and radiological aspects of this rare entity are discussed as well as the classification of this disease in the context of seronegative spondyloarthropathies.

Spontaneous healing of aneurysmal bone cysts. A report of three cases.

We report three cases of spontaneous healing of aneurysmal bone cysts (ABC). In one case histological material was obtained after resection of the already ossified expansile mass discovered as a lytic lesion seven months previously. In the two other patients, spontaneous ossification of a radiologically presumed ABC in the lytic and expansile phase was observed after nine and seven months respectively. The healed lesions have remained stable at 12, 32, and 36 months respectively. These findings suggest that when the diagnosis can be made with confidence, and the lesion is in a location and at a stage that does not entail any risk of fracture or compression, expectant management should be considered. Our three patients were aged 22, 19 and 18 years, older than usual for developing ABC. This is also true for many of the few other reported cases of spontaneous or almost spontaneous healing and suggests that ABC has a greater tendency to stabilise in older patients.

Adrenocortical function and responsiveness to tetracosactrin infusions after intra-articular treatment with triamcinolone acetonide and hydrocortisone acetate.

The urinary free cortisol (UFC) output, considered a parameter of the action of the exogenous corticosteroid on the hypothalamo-pituitary axis, was followed in five patients who received a total of 12 intra-articular (i.a.) injections of 40 mg of triamcinolone acetonide (TCA) distributed over eight treatment sessions. The effect of intra-articular TCA injections on UFC output was null after three treatment sessions, transient (48 hr) after another three, and more prolonged (6 days), although slight, after the remaining two sessions. These results contrast with the lasting and profound inhibition of the UFC output induced either by the same TCA dose given intramuscularly, or by oral administration of dexamethasone to 30 normal subjects, 0.75 mg at 8-hourly periods for 48 hours. Adrenocortical reactivity, as measured by the response to synthetic ACTH (tetracosactrin) of both plasma cortisol levels and UFC output, was studied in another five patients prior to and after eight i.a. injections of 40 mg of TCA, and in three patients after six i.a. injections of 25-100 mg of hydrocortisone acetate. No decrease was observed after the i.a. injections of HCA. Considered as a group the response to tetracosactrin after TCA injections was not significantly altered. Two patients showed a decreased response of both UFC and plasma cortisol, although their values remained within the limits of those obtained in 44 normal control subjects. In contrast, profound inhibition of the tetracosactrin induced response occurred in a control group of 30 subjects given 0.75 mg of dexamethasone orally at 8-hourly periods for 48 hours.

Adult onset of multifocal eosinophilic granuloma of bone: a long-term follow-up with evaluation of various treatment options and spontaneous healing.

We report a case of multifocal-monosystemic Langerhans cell histiocytosis (LCH), formerly usually referred to as eosinophilic granuloma (EG) of bone. The condition developed in a 36-year-old man. A notable infrequent thoracic spine location and two successive distinct costal lesions were observed. Both the first costal site and the vertebral location healed spontaneously; the second costal lesion underwent biopsy resection. The patient's disease course with an 8-year follow-up is discussed with reference to various treatment options, emphasising in selected cases a watchful conservative approach, in view of the widely documented potential for spontaneous healing.

Sciatic neuropathy at the popliteal fossa: clinical, ultrasonographic and computed tomographic diagnosis.

A 22-year-old female had been suffering from sciatica-like pain in the left leg for four years. Clinical findings strongly directed further investigations to the popliteal fossa. Ultrasonography located a hypoechogenic mass in the upper lateral popliteal space. Guided by these data, computerized tomography (CT) with vertical reconstructions made the tentative diagnosis of a common peroneal nerve tumor, which was confirmed at operation. Microscopic examination showed a neurinoma of the mixed neurilemmoma-neurofibroma type. In the presence of atypical features of sciatica, a high index of suspicion seems advisable. Emphasis is laid on the complementary contribution of ultrasonography and CT in this type of ill-defined lower limb pain.

Axillary schwannoma masquerading as cervical radiculopathy.

A 58-year-old woman, suffering from radicular-like pain in the left arm for 3 years, presented an entirely negative cervical imaging. Careful clinical examination disclosed Tinel's sign in the axilla. This clinical finding led to further investigation of this region. Computed tomography and magnetic resonance imaging disclosed a small tumour highly suggestive of a schwannoma. Surgical exploration and microscopic examination confirmed a diagnosis of schwannoma located on the radial trunk of the left brachial plexus. After tumour excision, the patient had immediate relief of pain without sensitive or motor sequelae. No recurrence has been observed after 3 years.

Abdominal wall weakness and lumboabdominal pain revealing neuroborreliosis: a report of three cases.

The authors report three cases of thoracic radiculoneuropathy disclosing neuroborreliosis. All three patients had low back and abdominal pain and two had marked abdominal wall paresis. EMG confirmed a motor involvement of the lower thoracic roots and CSF analysis revealed a lymphocytic meningitis in all three cases. Antibodies against Borrelia burgdorferi were present in both the serum and the CSF. A favourable outcome was obtained in all three patients with appropriate antibiotherapy. The differential diagnosis of this misleading presentation is discussed.

Paget's sarcoma--with two illustrative cases.

Two cases of Paget's sarcoma of bone are described. The first one is mainly characterized by the almost simultaneous degeneration of the only two existing locations of the underlying Paget's disease. The second case is one of monostotic Paget's disease of the scapula disclosed by the malignant degeneration.

The effect of low dosage glucocorticoids on bone mass in rheumatoid arthritis: a cross-sectional and a longitudinal study using single photon absorptiometry.

The cross-sectional study of patients with RA receiving LDGC, compared with those on NSAID alone (or patients with AS) showed that LDGC significantly affects bone mass at midshaft and even more so at the distal radius. The loss of bone seems to be brisk but continuous on the long run, at least at the distal scanning site, and thus increases the C/T ratio, especially in aged men. The loss of bone mass in the LDGC group correlates with the duration of the disease as well as with carpal destruction (especially at mid shaft radius), with both parameters being correlated with one another. At equal carpal destruction, LDGC still affects bone mass. Whether receiving NSAID alone or LDGC in addition, patients with RA, as compared with controls, are more liable to lose bone when they grow older. In a longitudinal study, premenopausal women were unaffected by the administration of LDGC at both scanning sites. In contrast, postmenopausal women receiving LDGC lost at least twice as much bone as did normal women after the menopause. Men of all ages on LDGC lost bone at a rate equal to that of normal women after the menopause. Men with RA or with AS on NSAID alone did not significantly lose bone. It is concluded that LDGC may be given to premenopausal women without harm to their bone mass. After the menopause, hormonal replacement therapy, if not contra-indicated, should be given in association with LDGC. Men fortunately have a higher peak bone mass and therefore can afford to lose bone during a decade before they attain the same situation as women at the time of their menopause. If treatment is then continued for another two decades, their bone mass might behave as does that of postmenopausal women if bone loss is continuous over such long periods of time. This latter assumption has yet to be verified.

Viral brachial neuritis in emergency medicine.

Brachial plexus neuritis is a rare neurologic disease that may be overlooked in emergency medicine because other conditions are much more common. We report a case of brachial plexus neuropathy due to cytomegalovirus infection. The diagnosis was based on history, clinical findings, laboratory tests, and electromyography. Early diagnosis and adequate treatment is important to avoid unnecessary investigation, prevent complications (especially adhesive capsulitis of the shoulder), and reassure the patient.

Novel nonsteroidal anti-inflammatory drugs.

The authors first briefly review how the concept of COX-2 selectivity was brought to light, then tested against the known gastrotoxicity ranking of currently used NSAIDs, from the old classics to the most recent. One truly selective COX-2 agent--celecoxib--is now being marketed in an ever increasing number of countries. So far it seems to keep its main promises, i.e. high--albeit not total--safety regarding gastrointestinal adverse effects, and undisturbed platelet function. Association with warfarin drugs seems to raise no problems, but one should still be wary of possible renal side-effects. Efficacy, at least as assessed in osteoarthritis and rheumatoid patients, appears satisfactory. However, treatment of intense inflammatory crises, such as gout or ankylosing spondylitis, has not been assessed, as yet. Another COX-2 agent--rofecoxib--is on the brink of being released. Its even more potent COX-2 selectivity raises new issues. What about some COX-1 activity that several authors detected in rheumatic synovitis? On the other hand, in particular circumstances, organs such as the stomach, the kidney and small blood vessels, seem to have their homeostasis partly controlled by COX-2 mechanisms also. These questions should be answered soon, whilst clinical experience with the COX-2 agent builds up.

Polymyalgia rheumatica. Assessment of disease activity using erythrocyte sedimentation rate and plasma viscosity.

Comparison of clinically assessed activity of disease with 112 paired readings of the erythrocyte sedimentation rate (ESR) and the plasma viscosity (PV) in 23 patients with polymyalgia rheumatica (PMR) showed the following. (1) A correlation between ESR and PV in both sexes, reaching the significance obtained in a comparison group of patients with rheumatoid arthritis (RA) (109 paired readings), with no significant difference between the PMR and RA groups on analysis of variance of the regression slopes. (2) A degree of scatter of readings around the regression lines so that they could not be used for prediction of ESR from the PV or vice versa. (3) varkappa(2) analysis of normal and abnormal values of ESR and PV which showed a highly significant correlation. However 10 readings were abnormally high for ESR in the presence of a normal PV. 5 of these 10 observations were associated with clinical features of disease activity. 20 readings were abnormally high for PV in spite of a normal ESR with only one instance of clinical activity. These data indicate that it is not possible to provide exact guidelines for a ;safe' level of ESR or PV applicable to the individual patient, and measurement of both these indices of disease activity is recommended.

Circulating immunoblasts in polymyalgia rheumatica.

The percentage of immunoblasts circulating in the peripheral blood has been examined in a group of 29 patients with polymyalgia rheumatica (PMR). Less than 0.5% of immunoblasts were found in healthy young controls, but 18 of 29 unselected patients with PMR were positive when first tested, a similar proportion to that found in rheumatoid arthritis. Raised immunoblasts were found in only one of 12 elderly controls. The presence of circulating immunoblasts correlated with the activity of polymyalgia both as assessed by the erythrocyte sedimentation rate (ESR) and as assessed by an independent clinical observer. This was true in the group overall and in those patients where serial studies were made. Patients studied from the time of disease presentation showed a concurrent fall in ESR and in immunoblasts on starting steroid therapy. Detection of circulating immunoblasts can be a useful additional test in the assessment of disease activity in PMR, especially in cases with a low ESR. The presence of circulating immunoblasts supports the concept of an immunological aetiology for PMR. This is strengthened by the finding that raised immunoglobulins were more common in patients with circulating immunoblasts.

Polymyalgia rheumatica. Abrupt and gradual withdrawal of prednisolone treatment, clinical and laboratory observations.

Eighteen patients with polymyalgia rheumatica had corticosteroid treatment withdrawn abruptly under close observation. In each case polymyalgic symptoms reappeared but were controlled rapidly when prednisolone was reintroduced. Prednisolone withdrawal was then started by slow decrements of dose. In no patient was it possible to withdraw prednisolone treatment, after using either method, during the period of observation.

Juvenile scleroderma and collagenous colitis. The first case.

We report the first case of coexisting progressive systemic sclerosis and collagenous colitis in a 12-year-old girl.

Primary prevention of glucocorticoid-induced osteoporosis with intermittent intravenous pamidronate: a randomized trial.

The aim of this study was to assess whether early intermittent I.V. administration of disodium pamidronate can effectively achieve primary prevention of glucocorticoid-induced osteoporosis (GIOP). A total of 27 in- or outpatients who required first-time, long-term corticosteroid therapy at a daily dose of at least 10 mg prednisolone were studied. Patients were randomly selected to receive either pamidronate and calcium or calcium alone. Patients allocated to pamidronate treatment (pamidronate group) received a first intravenous infusion of 90 mg pamidronate simultaneously with the initiation of their steroid treatment. Subsequently, they received 30 mg pamidronate, intravenously, every 3 months, for as long as steroid therapy was continued. As with the control patients (calcium group), they were put on a daily 800-mg elemental calcium supplement given as calcium carbonate. Lumbar spine and hip (total and subregions) bone mineral densities (BMDs) were measured at the start and every 3-months by dual-energy X-ray absorptiometry (Hologic(R) QDR-2000). Over 1 year, the pamidronate group showed a significant BMD increase in the lumbar spine (3.6%), and at all sites of the hip (2.2% at the femoral neck). In the calcium group, a significant BMD reduction was registered at the lumbar spine (-5.3%) and at the femoral neck (-5.3%). Differences between the groups were significant at all sites measured. Intermittent intravenous pamidronate effectively achieves primary prevention of GIOP, as assessed by BMD measurements over 1 year.

Monoarticular chronic synovitis in a child.

Lipoma arborescens is a villous lipomatous proliferation of the synovial membrane characterized by chronic and painless synovial effusion. The aetiology is unknown. It has to be included in the differential diagnosis of chronic monoarticular disease in childhood. Magnetic resonance imaging provides a highly efficient tool for the diagnosis of this very rare condition. This is indeed the fourth paediatric case reported. Rather than resorting to the often inconvenient surgical synovectomy commonly recommended, we chose to treat the knee of this 13-yr-old boy with intra-articular osmic acid.