Adult onset Still's disease: review of 41 cases.

OBJECTIVES: To describe the clinical, laboratory and radiological features, treatment and prognosis of patients with adult onset Still's disease (AOSD). METHODS: Specific clinical features were retrospectively recorded in 41 patients fulfilling the Yamaguchi criteria. Patients were reviewed in two academic hospitals with a referral area of 700,000-1,000,000 inhabitants. Laboratory tests including haemogram, ferritin, biochemistry and autoimmunity were reviewed. Radiological studies, treatment and ACR functional class were determined. RESULTS: Forty-one patients with AOSD were identified, 25 of whom were female. Mean age at diagnosis: 38.19 years (range 17-68). Feverish polyarthritis was the most common clinical presentation. Acute phase reactants were invariably high in all patients. Serum ferritin levels were elevated in 86% of patients. Anti-cyclic citrullinated peptide antibodies (anti-CCP antibodies) were negative in all patients except one. The course of the disease was monocyclic in 44% of the patients, polycyclic in 26%, and chronic articular in 30%. ACR class was as follows: 29 (72.5%) class I, 7 (17.5%) class II, 2 (5%) class III and 2 (5%) class IV. As for the treatment received, aspirin or NSAIDs controlled the disease in eight patients (19.5%) and high-dose corticosteroids (0.5-1 mg/kg/day) in 32 (78%). Almost half of the patients (49%) required an additional diseasemodifying agent, usually methotrexate. Finally, in seven of them (17%) a biological treatment with TNF-α or specially anti-IL-1 had to be added to control the disease. CONCLUSIONS: The clinical and laboratory findings were similar to previous studies. Anti-CCP antibodies were almost always negative. A monocyclic course was associated with a good prognosis. Most of the patients were in ACR functional class I and II. Biological agents were required in 7 patients (17%).

Correlation between optical coherence tomography and glaucomatous optic nerve head damage in children.

AIM: To compare analysis of macular and nerve fibre layer thickness by optical coherence tomography (OCT) with optic nerve head (ONH) morphology based on stereophotography. DESIGN: Prospective observational case-control series. METHODS: Normal and glaucomatous eyes of children (age 4-17 years) were scanned using Stratus OCT (Carl Zeiss Meditec, Dublin, California, USA). Fast macular and retinal nerve fibre layer (RNFL) thickness map were performed on 372 eyes of 222 children. ONH stereophotographs were taken and evaluated by two masked observers using a grading system of 0 to 5 based on both cupping ratio and morphology. OCT3 analyses were compared across ONH grades for different areas around the macula and the peripapillary RNFL. RESULTS: Analysis included OCT values and ONH grading for 139 eyes of 139 children. There was a negative correlation between ONH grade and both macular thickness and RNFL thickness in all areas measured. There was a difference in the correlation identified for black versus white children. CONCLUSION: OCT measurements of RNFL and macular thickness declined with increasing grade of glaucomatous damage seen on stereophotographs in black and white children. Further study will help quantify the value of OCT in the diagnosis and management of paediatric glaucoma.

Lack of deleterious effect on bone mineral density of long-term thyroxine suppressive therapy for differentiated thyroid carcinoma.

The effect of subclinical hyperthyroidism on bone mineral density is controversial and could be significant in patients with differentiated thyroid carcinoma who receive suppressive doses of levothyroxine (LT4). To ascertain whether prolonged treatment with LT4 to suppress thyrotropin had a deleterious effect on bone mineral density and/or calcium metabolism in patients thyroidectomized for differentiated thyroid cancer we have performed a cross-sectional study in a group of 88 women (mean +/- SD age: 51 +/- 12 years) treated with LT4 after near-total thyroidectomy and in a control group of 88 healthy women (51 +/- 11 years) matched for body mass index and menopausal status. We determined calcium metabolism parameters, bone turnover marker N-telopeptide and bone mass density by dual-energy X-ray absorptiometry. No differences were found between patients and controls in calcium metabolism parameters or N-telopeptide except for PTH, which was significantly increased in controls. No differences were found between groups in bone mineral density in femoral neck (0.971 +/- 0.148 gr/cm(2) vs 0.956 +/- 0.130 gr/cm(2) in patients and controls respectively, P = 0.5). In lumbar spine, bone mineral density values were lower in controls than in patients (1.058 +/- 0.329 gr/cm(2) vs 1.155 +/- 0.224 gr/cm(2) respectively, P < 0.05). When premenopausal (n = 44) and postmenopausal (n = 44) patients were compared with their respective controls, bone mineral density was similar both in femoral neck and lumbar spine. The proportion of women with normal bone mass density, osteopenia and osteoporosis in patient and control groups was similar in pre- and postmenopausal women. In conclusion, long-term suppressive LT4 treatment does not appear to affect skeletal integrity in women with differentiated thyroid carcinoma.

Paget's disease of bone in early adult life.

OBJECTIVE: To determine the range and activity of the clinical and biological features of patients aged <40 with Paget's disease of bone. METHODS: A retrospective two centre study of 314 patients with Paget's disease of bone from two university hospitals. The disease was diagnosed by radiological, serum alkaline phosphatase (AP) levels, or clinical features, and bone scintigraphy in most patients. Demographic data, reason for diagnosis, bones affected, disease extent using Coutris' index, complications during progression, and disease activity using Renier's index were assessed. Patients over and under 40 were compared. RESULTS: 18/314 (5.7%) patients were diagnosed before the age of 40; median (SD) age was 35.4 (5.5) (range 18-40) and AP 555.6 (566.3) IU/l (range 70-1949). Coutris' extension index was 12.8 (10.5) and Renier's activity index 35.9 (31.9). Younger patients had more affected bones (p<0.05) than those aged >40, higher level of extension (p<0.05), higher AP value (p = 0.05), and greater incidence of thoracolumbar spine disease. Disease activity did not differ significantly between the groups. CONCLUSIONS: Paget's disease diagnosed before the age of 40 is more extensive but not more active, with higher AP values than in those diagnosed after age 40.

Treatment intensity significantly influencing fibrosis in bone marrow independently of the cytogenetic response: meta-analysis of the long-term results from two prospective controlled trials on chronic myeloid leukemia.

Bone marrow fibrosis (MF) has been shown to indicate therapy failure in Ph(+) chronic myeloid leukemia (CML). However, the results on the development of MF during interferon-alpha therapy of CML are controversial. The significance of the interferon dose has not been considered as yet. In total, 627 bone marrow biopsies taken prospectively from 200 patients with CML recruited in two studies using different doses of interferon-alpha +/- low-dose cytosine arabinoside were examined for MF before and during therapy. The results showed that the risk of MF depended significantly on the interferon-alpha dose applied (P<0.000005). MF progressed during low-dose therapy (3 x 5 x 10(6) IU/week), but was prevented from progression when applying high dose (5 x 10(6) IU/m(2)/per day). MF disappeared when high-dose interferon-alpha was combined with low-dose cytosine arabinoside (P<0.000005). The risk of death markedly increased when MF occurred or progressed (P<0.0009), independent of all other prognostic factors evaluated including the cytogenetic response. In conclusion, the effectiveness of interferon-alpha on MF depends on the treatment intensity. MF reverses when combining high-dose interferon-alpha with low-dose cytosine arabinoside, but progresses when applying low-dose interferon-alpha. MF appears to be a significant early indicator of ineffective therapy in CML.

Enfermedad de Still del adulto en una gestante: tratamiento y evolución / Still´s disease in a pregnant woman: treatment and outcome

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Association of systemic lupus erythematosus and hypermobility.

OBJECTIVE: To investigate joint laxity in patients with systemic lupus erythematosus (SLE). SETTING: University Hospital. METHODS: 81 patients with SLE (1999 American College of Rheumatology criteria; 72 (89%) women and nine (11%) men, mean age 42.9 (SD 16.1) years), who regularly attended a specialist SLE clinic were examined. The control group comprised 280 patients attending a general practitioner (193 (69%) women and 87 (31%) men, mean age 44.7 (SD 11.2) years). Joint laxity was measured according to the criteria of Beighton et al (total score 4 or more). A regression analysis was performed. RESULTS: Thirty nine (48%) patients with SLE and 42 (15%) of the control group were hypermobile. A logistic regression model was developed. The odds ratio of the association between laxity and SLE after adjustment for age and sex was 2.31 in the group younger than 49 years, and 17.99 in the group aged 49 years or older. Neither the clinical and analytical profile nor the use of corticosteroids was related to joint laxity. CONCLUSION: Patients with SLE showed more hypermobility than controls. Hypermobility was more profound in older patients with SLE (> or =49 years). Joint laxity was not associated with any clinical or analytical pattern. Treatment with corticosteroids was not related to joint laxity.

Linfadenitis necrosante de Kikuchi-Fujimoto y su asociación con el lupus eritematoso sistémico: descripción de 4 nuevos casos / Kikuchi-Fujimoto necrotizing lymphadenitis and its association with systemic lupus erythematosus: description of 4 new cases

Objetivo: Describir las características clínicas de la linfadenitis necrosante de Kikuchi Fujimoto y evaluar su posible asociación con el lupus eritematoso sistémico. Métodos: Diseño retrospectivo. Se revisaron las historias clínicas de aquellos casos de linfadenitis de Kikuchi Fujimoto y lupus eritematoso sistémico que habían sido visitados en la Sección de Reumatología del Hospital Universitari Germans Trias i Pujol durante los años 1984-2001. Resultados: Se identificaron 4 casos de linfadenitis de Kikuchi: 4 mujeres (media de edad, 31 años; límites, 27-34). En todos ellos se llegó al diagnóstico a partir de la biopsia ganglionar. Tres de los casos estaban asociados a lupus eritematoso sistémico. Conclusión: La linfadenitis necrosante de Kikuchi Fujimoto parece estar asociada con el lupus eritematoso sistémico. (AU)

Musculoskeletal manifestations in patients positive for human immunodeficiency virus: correlation with CD4 count.

OBJECTIVE: To determine the relationship between the CD4+ lymphocyte count and musculoskeletal manifestations of human immunodeficiency virus (HIV) infection. METHODS: All patients from 1991 to 1998 who were positive for HIV with osteoarticular manifestations were reviewed retrospectively. HIV positivity was confirmed by ELISA and Western blot. CD4 count was performed by flow cytometry. RESULTS: We studied 74 patients with osteoarticular manifestations. The study group comprised 61 men (82.4%) and 13 women (17.5%) with a mean age of 34.2 years (range 17-62). Fifty-two patients were iv drug users (70.3%). Septic arthritis was present in 20 cases (23.0%), soft tissue infections in 9 cases (12.2%), spondyloarthropathies in 6 cases (8.1%), lymphomas in 9 cases (12.2%), osteomyelitis in 6 (8.1%), and 24 miscellaneous cases (32.4%). The mean CD4 count was as follows: septic arthritis 164.7 cells/mm3, soft tissue involvement 127.1 cells/mm3, spondyloarthropathies 245.8 cells/mm3, lymphoma 132.8 cells/mm3, and osteomyelitis 233.6 cells/mm3. CONCLUSION: Osteoarticular manifestations in the setting of HIV infection tend to be predominantly infectious. S. aureus is the microorganism most frequently involved. Ostearticular infections always appeared when the CD4 count was < 200 and pyomyositis and lymphoma appeared when CD4 was < 150. CD4 counts may be useful predictors to determine the type of musculoskeletal manifestation.

Aseptic spondylodiskitis in rheumatic diseases.

Aseptic diskitis is relatively common. Several rheumatic diseases involving the spine may have this complication. As this condition mimic infectious diskitis, it is important to recognize it. Clinically, it is characterised by vertebral pain of an inflammatory nature, occasionally accompanied by fever and an increase in the erythrocyte sedimentation rate. Radiologically, the decrease in the articular space and the irregularity of the vertebral plates are of particular importance. Although uncommon, it is necessary to bear in mind this possibility when the etiological search for a possible infectious diskitis proves fruitless. A MEDLINE (1986-2000) and PUBMED (1966-2000) search of relevant articles was performed. Descriptors used were aseptic diskitis, spondylodiskitis, pseudodiskitis and pseudoarthrosis.

Achilles tendinitis as the presentation form of Löfgren's syndrome.

Löfgren's syndrome is characterised by bilateral hilar adenopathy arthritis and erythema nodosum. Achilles tendinitis as the presentation form of Löfgren's syndrome is very unusual. Herein we present a case of bilateral achilles tendinitis as the presentation form of Löfgren's syndrome.

[Front chest pain and paresis of recurrent nerve: sapho syndrome]. / Dolor torácico anterior y paresia del nervio recurrente: síndrome sapho.

The AA. present a rarely encountered case combining a left recurrent palsy due to an sclerosis and hyperostosis of the first costoesternal joint, which improved after an antiinflammatory treatment.

[The SAPHO syndrome: a study of 16 cases]. / Síndrome SAPHO: estudio de 16 casos.

The SAPHO syndrome is characterized by synovitis, acne, palmo-plantar pustulosis, hyperostosis and osteitis. SAPHO syndrome has been occasionally described in Spain. We present our experience of 16 cases with the SAPHO syndrome diagnosed between 1984-1995. The predominant clinical symptoms were: anterior thoracic pain (14 cases), sacroiliac pain (7 cases) and peripheral arthritis (2 cases). Cutaneous involvement was characterized by palmo-plantar pustulosis (8 cases) acne (3 cases) and psoriasis (2 cases). The histocompatibility antigen B27 was negative in all cases. A computerized tomographic study revealed involvement of sterno-costo-clavicular and manubriosternal joints (14 cases) and sacroiliitis (8 cases).