Sandra Trapani

Osteoporosis in juvenile systemic lupus erythematosus: a longitudinal study on the effect of steroids on bone mineral density

Abstract Peak bone mass is an important determinant of future bone mass and of the risk of osteoporosis and subsequent fractures. Although some information concerning bone mineral density (BMD) in adults affected with systemic lupus erythematosus (SLE) is available, few data on children and adolescents have been reported. Many variables, such as duration and activity of the disease, reduced sun exposure, and steroid therapy have been suggested as risk factors in the pathogenesis of osteoporosis in SLE. In this study, we longitudinally evaluated, by dual energy X-ray absorptiometry (DEXA), the BMD of 20 young patients affected with juvenile SLE (JSLE), in order to establish the degree of osteoporosis and the influence of steroid treatment, among other clinical variables. At baseline, the mean BMD in JSLE patients was 0.978 g/cm2 and in controls 1.038 g/cm2 (P=0.31). At 1 year (time 2), this value became 0.947 g/cm2 in JSLE children; the mean individual difference was 0.28 g/cm2 (3.4%). Only in those patients aged 19–25 years BMD was significantly lower than in controls, both at baseline and at time 2. Considering the steroid treatment, no significant difference between the two groups was found either at baseline or at time 2; however, the mean yearly BMD loss in the steroid patients was 0.031 g/cm2 (3.5%) vs. 0.005 g/cm2 (0.5%) in those who had not taken steroids. A significantly inverse correlation between BMD and the cumulative dosage of corticosteroids has been detected. BMD produced a significantly inverse correlation to the cumulative dosage of corticosteroids; no significant correlation has been found between BMD and disease activity or duration.

Human parvovirus B19 infection: its relationship with systemic lupus erythematosus

OBJECTIVES The clinical presentation and outcome of four cases of human parvovirus-B19 (HPV-B19) infection, initially diagnosed as systemic lupus erythematosus (SLE), were reviewed and compared with similar cases previously reported in the literature. The relationship between HPV-B19 infection and SLE is discussed. METHODS The medical records of four patients with documented HPV-B19 infection, initially diagnosed as SLE, were reviewed and studied in detail. A Medline search from 1985 to 1997 was performed to identify other cases reported in the literature in which a relationship between HPV-B19 and SLE had been identified in both adults and children. RESULTS In all of our cases, the clinical findings (fever, rash, arthritis and malaise) and hematologic data (leukopenia, thrombocytopenia, anemia, presence of autoantibodies, hypocomplementemia, etc.) had initially suggested a diagnosis of juvenile SLE. Subsequently, evidence of HPV-B19 infection at the time of clinical presentation was ascertained. In three of these cases, the disease course was self-limiting with complete clinical remission and normalization of hematologic abnormalities within 18 months; one case, however, had persistent disease activity and repeated exacerbations. CONCLUSIONS The occurrence of HPV-B19 infection has been documented in patients with SLE, in particular in relation to disease onset. Similarities in clinical and immunological features of viral infections and SLE at presentation may hinder the differential diagnosis between these two conditions. The family history, a self-limiting disease course and certain disease specific clinical aspects may help the pediatrician formulate an accurate diagnosis. In our patients, HPV-B19 infection may have mimicked the onset of SLE in three cases, but triggered the disease in one.

Correlation between clinical and ultrasound assessment of the knee in children with mono-articular or pauci-articular juvenile rheumatoid arthritis

Background. Ultrasonography of the knee is a non-invasive, readily available and low-cost tool for demonstrating peri-articular tissues. Objective. To correlate clinical features with US findings in the detection, quantification and follow-up of inflammatory signs of the knee in children with pauci-articular juvenile rheumatoid arthritis (JRA). Materials and methods. US of both knees was performed in 49 patients on the same day as the clinical examination. All joints were classified into two groups by clinical criteria: group A (active disease) or group B (quiescent disease). Thirteen patients underwent one or more follow-up examinations. US was performed with a small-parts, 7.5-MHz, electronic linear probe by using a technique previously reported. Quantitative assessment of any effusion and synovial thickening was evaluated at the level of the suprapatellar bursa. Wilcoxon and Spearman tests were employed to compare US findings between the two groups and to correlate clinical and US findings within each group, respectively. Results. US demonstrated significant increase of effusion and synovial thickening in group A joints. US enabled visualisation of clinically undetected popliteal cysts in three patients. Correlation between clinical and US findings was significant in group A and positive, though not significant, in group B. Conclusions. US seems to be a sensitive and reliable method for the assessment and monitoring of knee joint involvement in pauci-articular JRA.

Intravenous administration of alendronate counteracts the in vivo effects of glucocorticoids on bone remodeling.

The purpose of the investigation was to test the use of alendronate in the therapy of children affected by chronic rheumatic diseases and symptomatic drug-induced osteoporosis. Two courses of alendronate were intravenously administered to four girls with vertebral fractures that were glucocorticoid induced. Improvement of back pain and bone mineral density increase evaluated by DXA and conventional spine X-rays were observed. Our study supports the ABD-induced improvement of the negative effects of long-term therapy on bone mineral density in children with chronic rheumatic diseases.

The primary role of steroids on the osteoporosis in juvenile rheumatoid arthritis patients evaluated by dual energy X-ray absorptiometry

Osteoporosis, a common clinical feature in children affected with Juvenile Rheumatoid Arthritis (JRA), is generally divided into two forms, a localized juxta-articular osteoporosis of the single joints and a generalized reduction of bone mass due to the disease itself, joint involvement and steroid treatment. Recently Dual Energy X-ray Absorptiometry (DEXA) has been suggested for Bone Mineral Density (BMD) measurement. In the present study DEXA has been used to investigate the lumbar spine BMD in JRA patients as compared to healthy children. Our results showed that BMD is reduced in JRA patients (BMD 0.685 g/cm2) when compared to healthy children (BMD 0.722 g/cm2), and it is significantly lower in the group of patients treated with steroids (BMD 0.623 g/cm2) when compared to those treated with nonsteroidal antiinflammatory drugs (BMD 0.710 g/cm2). The analysis of all risk factors of bone loss indicated that steroids represent the only parameter of significant negative correlation with BMD.

Effect on lung function of methotrexate and non-steroid anti-inflammatory drugs in children with juvenile rheumatoid arthritis

Abstract We evaluated lung function in a group of patients affected by juvenile rheumatoid arthritis (JRA), without clinical and/or radiological signs of respiratory involvement. We compared the effects on pulmonary function of methotrexate (MTX) therapy combined with non-steroid anti-inflammatory drugs (NSAIDs) to those of NSAIDs alone and correlated lung function to subtype onset, disease duration and disease activity. Our patients were 27 JRA children, subdivided into two groups according to the therapy (group A=14 patients, treated with a low dose of MTX and NSAIDs; group B=13 patients, treated with NSAIDs alone). Clinical evaluation, haematological data and pulmonary function tests (PFTs) were obtained in each group at baseline (time 0) and at 1 year (time 1). At time 0 and time 1 PFTs were altered in 51.8% of JRA patients. The restrictive pattern (reduced forced vital capacity, FVC) was the most frequent feature, observed in 22.2% of patients. In group A the mean values of FVC, FEV1 (forced expiratory flow in 1 s), FRC (functional residual capacity), TLC (total lung capacity) and DLCO (diffusing lung capacity of carbon monoxide) were significantly lower compared to those of group B, at time 0 and at time 1. No functional parameter was correlated to subtype, duration or activity of the disease. Our study confirms that abnormalities in PFTs may be detected in JRA patients, even in the absence of clinical and/or radiological signs of lung disease; MTX in combination with NSAIDs does not seem to affect lung function at 1 year more than NSAIDs alone.

Myositis ossificans circumscripta: a paediatric case and review of the literature.

Myositis ossificans circumscripta (MOC), characterised by non-neoplastic heterotopic bone formation in soft tissue and skeletal muscle, is rare in children. At onset, it is difficult to distinguish MOC from a musculoskeletal infection or neoplasm, particularly in absence of trauma, and a biopsy is frequently required. We describe an 11-year-old boy with MOC in his thigh. At admission, minor local trauma was referred, and clinical examination revealed a tender and painful mass in the left thigh. Hypothesising a periarticular infection, a wide-spectrum antibiotic and an anti-inflammatory drug were given. The magnetic resonance imaging revealed an enlarged mass with marked enhancement of the lesion and a central, irregular non-enhanced area due to necrosis. After incisional biopsy, the histopathological examination found immature osteoblasts inside striated muscle fibres, as well as proliferating fibroblasts, which are all compatible with MOC, thereby ruling out infection or malignancy. The CT scan showed calcified deposits arranged in a ring, thus confirming MOC; a radiological follow-up was suggested to detect the lesion’s maturation. Three months after diagnosis, surgical excision was performed. The post-operative period was uneventful. Six months later, the child was in good condition, with no signs of recurrence. In the literature, 57 paediatric cases have been described; most of these had an acute course, required excision and then had a favourable evolution. Our case study confirms the good prognosis of MOC and underlines how this benign condition should be considered in children presenting a tender and painful soft-tissue swelling.

Severe hemorrhagic bullous lesions in Henoch Schonlein purpura: three pediatric cases and review of the literature

Henoch Schonlein purpura (HSP) is a small-sized blood vessels vasculitis, resulting from immunoglobulin A-mediated inflammation. It is the most common acute systemic vasculitis in childhood and mainly affects skin, gastrointestinal tract, joints and kidney. Skin lesions, usually presenting as erythematous maculopapules, petechiae, and purpura, often involve lower extremities and buttocks, but may also extend to the upper extremities, face and trunk. Conversely to adults, hemorrhagic bullous evolution has been seldom described in childhood. The pressure is likely a factor into the pathogenesis of bullae. We report on three new pediatric cases of HSP with hemorrhagic bullous skin lesions, and a review of the literature. Bullous evolution represents an unusual, but well-recognized cutaneous manifestation that may be a source of diagnostic dilemma, but does not seem to have any prognostic value in the outcome of HSP.

Salmonella pelvic osteomyelitis in an immunocompetent child

A case of Salmonella enteritidis osteomyelitis isolated from the pelvis in a healthy adolescent is reported. Pelvis osteomyelitis is rare in childhood. Moreover, salmonella, typically responsible for bone infection in sickle cell anaemia, is unusually involved in healthy children. Salmonella should be considered among aetiological agents. Antibiotic treatment alone may be successful; however, sometimes, surgical drainage is required.

An 18‐month‐old child with seizures and bloody diarrhea

To the Editor: An 18 month-old infant with stupor status was admitted to the local hospital in April 2002. Three months earlier, he had presented with occasional mucus and fresh blood in his stool. One week prior to hospital admission, he begun to experience progressively worsening paleness, fatigue, and bloody diarrhea with mucus, and had an episode of tonicclonic fits of the right arm lasting a few seconds. On hospital admission, a blood analysis revealed the following: hemoglobin concentration, 3.6 mg/dL; platelet count, 748,000/mm; and C-reactive protein concentration, 2.9 mg/dL. A lumbar puncture was performed, revealing normal cerebral spinal fluid. During a blood transfusion, the child experienced a generalized seizure with loss of consciousness and upward deviation of his eyesight. Computerized axial tomography, magnetic resonance (MR) imaging, and MR angiography scans of the brain revealed bilateral hemorrhagic infarctions of the basal ganglia, and thrombosis of the internal cerebral veins, Galeno’s vein, rectum, and left transverse sinus (Fig. 1). Blood flow was preserved in the superior sagittal sinus and the right transverse sinus. The child was then transferred to the pediatric intensive care unit of our hospital where a physical examination revealed a suffering, pale, dehydrated child, who was in opisthotonus without signs of meningism. Some type of encephalitis was initially suspected, and therapy with antibiotics, steroids, acyclovir, aspirin, and phenobarbitone was started. Because of intestinal bleeding, anticoagulants were not administered. As soon as his clinical condition was stabilized, he was transferred to our ward. His neurologic condition improved, but bloody diarrhea with mucus was still present up to 7 to 10 times a day. Prothrombin time, partial thromboplastin time, plasma levels of protein C and protein S, and resistance to activated protein C and antithrombin III were compatible with endothelial distress. The Leyden factor V mutation and the G20210A mutation of factor II were absent, although the patient was etherozygous for the methyltetrahydrofolate reductase (C677T) mutation. The plasma concentrations of homocysteine, vitamin B12, and folate were normal. The results of immunologic tests, such as those for anticardiolipin antibodies and lupus anticoagulants, were normal, but test results for antineutrophil cytoplasmic antibodies with a perinuclear pattern were positive. The findings of a rectosigmoidoscopy with biopsy and histopathologic examination were consistent with the diagnosis of ulcerative colitis (UC). The patient was started on a treatment regimen of broad-spectrum intravenous antibiotics, intravenous methylprednisolone, and total parenteral nutrition. After 9 days of treatment, he was still presenting with bloody diarrhea, and a blood transfusion was required. For this reason, intravenous cyclosporine (3 mg/kg/d) was added to therapy, and then continued orally (7 mg/kg/d) with 6 mercaptopurine. At this stage, an MR imaging scan of the brain and abdomen was performed, and it revealed the complete recanalization of the cerebral vessels, although irregular appearances of the sigmoid colon and rectum were detected (Fig. 1). Despite such intensive medical treatment, the child continued to have bloody diarrhea with slowly declining hemoglobin levels, which required a blood transfusion. Therefore, a total colectomy with ileoanoanastomosis and protective ileostomy was performed 3 months after hospital admission. The macroscopic and histologic appearance of the colon confirmed the diagnosis of UC. During the postoperative course, the patient initially required therapy for severe electrolyte disturbance. His clinical condition then improved, and consequently oral feeding was reintroduced and total parenteral nutrition was stopped. The ileostomy was closed after 1 year. At the 2-year follow-up, the child was growing well. From a neurologic perspective, however, ambulation and speech deficits were present, and he developed postischemic epilepsy. Thromboembolism (TE) is a potentially life-threatening event that is associated with inflammatory bowel disease (IBD). A population-based cohort study has recently shown that IBD patients have a 3-fold increased risk of developing deep venous thrombosis and pulmonary embolism compared with the general population. IBD seems to be a specific risk factor for TE as neither rheumatoid arthritis, another chronic inflammatory conditions, nor celiac disease, another chronic bowel disease, carry an increased risk of TE. IBD paFigure 1. MR imaging scan of the brain (A) and colon (B). A T1-weighted image of the brain showing thrombosis (arrow) of the rectum sinus (A). A T2-expressed sequence showing the irregular shapes of the sigmoid colon and rectum (B).