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ANALISI DI NUOVI MARCATORI CELLULARI E MOLECOLARI PER LA SPONDILITE ANCHILOSANTE
No full textObjective. Here we investigated spontaneous and induced osteoclastogenesis in peripheral blood
mononuclear cells (PBMCs) from ankylosing spondylitis (AS) patients, in order to explore the
relation between osteoclastogenesis and disease activity. In addition, in order to explore functional
characteristics of AS osteoclasts (OCs), we tested their susceptibility to undergo apoptosis
investigating their response to Emblica officinalis plant extracts, previously characterized by us as
apoptotic inducer in OCs.
Methods. PBMCs from healthy individuals and AS patients were cultured with or without
osteoclastogenic inducers, stained for tartrate-resistant acid phosphatase (TRAP), and
immunostained for Bcl-XL, Survivin and Bim proteins. After treatment with E. officinalis extracts,
OCs were subjected to TUNEL test for measuring apoptosis. Disease activity was measured by
Ankylosing Spondylitis Disease Actvity Score-CRP (ASDAS-CRP) and magnetic resonance
imaging.
Results. We found that PBMCs from AS are more susceptible to spontaneous osteoclastogenesis
than the PBMCs from control samples, and that the osteoclastogenic potential was significantly
associated with high ASDAS among AS patients with bone marrow edema. In addition, after
treatment with E. officinalis extracts normal OCs underwent apoptosis, but OCs from AS patients
showed a strong resistance to apoptotic stimulus. We found that OCs from AS patients, unlike OCs
from healthy individuals, express high levels of survival signals including Bcl-XL and Survivin, but
do not express Bim proapoptotic protein.
Conclusion. Our data demonstrate that AS OCs present qualitative peculiarities: this may help in
identifying an effective strategy to prevent bone changes in AS
Adult-onset Still's disease: still a long way to go. Comment on the article 'Adult-onset Still's disease: review of 41 cases' by Riera et al.
No full textSirs,
We have read with great interest the article
by Riera et al., describing clinical and laboratory
characteristics of a large cohort of patients
with adult-onset Still disease (AOSD),
recently published in Clinical and Experimental
Rheumatology (1).
We agree that serum ferritin levels are very
useful both to diagnose and to monitor patients
with AOSD. Our work, previously
published in Seminars in Arthritis and Rheumatism
suggests that ferritin may be a useful
prognostic factor, perhaps in combination
with others (2). Interestingly, as shown in
Table I, comparing serum ferritin levels in
patients with chronic systemic and chronic
articular disease at the time of diagnosis and
6 months later, it is possible to infer that the
persistence of high ferritin levels, after adequate
treatment may predict a chronic articular
course.
Our experience with anti-TNF-α agents, partially
presented at the 2007 ACR meeting, is
also corroborated by that of Riera et al. Anti-
TNF-α medicines, in fact, may be helpful in
controlling systemic and articular symptoms
of refractory AOSD. Our data have suggested
that TNF-α blockade would not be as effective
in slowing down the articular damage
as in improving systemic and osteo-articular
symptoms This leads to the awareness that
anti-TNF-α agents should not be a conceivable
first line therapy in AOSD. Owing to a
more reassuring safety profile and a more targeted
action, as well as more evidence-based
effectiveness, we think that interleukin-1
receptor antagonist anakinra could actually
be considered a first line choice in AOSD
patients (3, 4).
Although AOSD has usually been considered
a benign disease, we think it is worth pointing
out that patients with the systemic form of the
disease should be closely monitored because
of the possibility of life-threatening complications,
that could also represent the heralding
manifestation of the disease (5-8).
In conclusion, we think that a multi-centre
study aimed at the identification and validation
of a set of diagnostic criteria should be
encouraged. There are currently several classification
criteria for AOSD, but those of
Yamaguchi are the most used (9). However, it
is worth recalling that the latter are not specified
in detail; in particular the exclusion criteria
provide only general recommendations
and there is no precise list of diagnoses or
set of laboratory tests to be performed. The
second important limitation is that serum ferritin
and its glycosylated component are not
included as is in Fautrel’s proposed criteria
(10). Besides, Yamaguchi’s criteria give the
possibilty to classify AOSD patients as having
no fever. As far as fever is concerned, we
think that a feverish polyarthritis should be a
sine qua non criteria for suspecting a diagnosis
of AOSD. Larger, prospective studies are
also needed to identify reliable prognostic
factors
Fatal myocarditis in adult-onset Still disease with diffuse intravascular coagulation
No full textAdult-onset Still disease (AOSD) is a rare condition disease of unknown etiology, characterized by quotidian or double quotidian spiking fever, with an evanescent pink-salmon rash, arthritis and multi-organ involvement. Diagnosis is usually clinical and made after other diseases in the differential diagnosis are excluded. We herein report the case of a patient with a remarkable familial autoimmune background in whom adult Still disease started off with a diffuse intravascular coagulation, probably triggered by a macrophage activation syndrome, followed by an acute interstitial myocarditis, leading to a fatal complete atrioventricular block. This case highlights that AOSD represents a troubling condition and that it may suddenly get worse with life-threatening events
La sindrome SAPHO: una entità clinica ancora poco conosciuta. SAPHO syndrome: a still little known clinical syndrome.
No full textLa sindrome SAPHO è caratterizzata dall'associazione, anche non simultanea, di manifestazioni osteo-articolari e cutanee. Il quadro articolare è caratterizzato soprattutto dal coinvolgimento della parete toracica anteriore. Vengono prese in rassegna le metodiche strumentali più idonee per identificare le lesioni e l'atteggiamento terapeutico corrente
Sustained remission of SAPHO syndrome with pamidronate: A follow-up of fourteen cases axnd a review of the literature
No full textObjective. To evaluate the efficacy of intravenous (i.v.) pamidronate in patients with SAPHO syndrome refractory to first line treatments and to review the available literature on pamidronate for this indication.
Methods. We report 14 cases of SAPHO syndrome refractory to non-steroideal anti-inflammatory drugs (NSAIDs), glucocorticoids and disease modifying anti-rheumatic drugs (DMARDs) treated with i.v. pamidronate. All patients received i.v. 60 mg pamidronate/day for 3 consecutive days. The primary evaluation criterion was the disappearance of bone pain, considered as the reduction in the visual analogic scale for pain (VAS) greater then 50 %.
Results. Ten patients were females and 4 were males. The mean age at onset was 40.4 years old. Ten patients presented a relapsing-remitting course, while in 4 cases the disease followed a prolonged course. In all cases anterior chest wall involvement occurred early in the disease. In 2 cases there was also a peripheral monoarthritis. Eleven patients experienced several flares of palmo-plantar pustulosis, while severe acne was present in 2. In a case there was no cutaneous involvement. Twelve of the 14 patients had a good response after 3 infusions and in 8 of these patients a sustained remission was observed. The recurrence of skin manifestations does not seem to be influenced by pamidronate.
Conclusions. Pamidronate appears to be an effective treatment in the osteo-articular manifestations of SAPHO syndrome. As far as cutaneous lesions are concerned, evidence of efficacy is not so impressive
Pamidronate and zolendronic acid in the treatment of Paget’s disease of bone.
No full textAbstract: Paget’s disease of bone (PDB) is a condition characterized by excessive and abnormal bone remodelling. Due to a high rate of bone remodelling, bisphosphonates, and especially pamidronate and the newer zolendronate, are indicated in the treatment of PDB. The presence of asymptomatic, but active PDB represents an indication for treatment aimed at preventing later complications.
An additional indication for treatment is the involvement of skeletal segments that may give rise to severe complications. Pamidronate has a long history in the treatment of PDB. The more utilised regimen is 3 to 6 i.v. infusion of 60 mg of pamidronate at an infusion rate of 1 mg/min within 3–21 days. Zolendronate (5 mg once yearly) is the most powerful amino-bisphosphonate currently used. This primacy recognizes both the ability to inhibit the farnesyl-pyrophosphate synthetase and the higher affinity to hydroxyapatite crystals as a cause.
Both pamidronate and zolendronate are effective in PDB, with an evidence-based superiority of the latter.
Keywords: Paget’
Impostomous gout
No full textIn older people, gout has equally incidence in both sexes.
• In older people, gout is usually polyarticular insidious
involvement at onset.
• In older people, gout involves any joints and tophy is
common at presentation
Precision Medicine in Rheumatology: The Role of Biomarkers in Diagnosis and Treatment Optimization
No full text: Rheumatic diseases encompass a wide range of autoimmune and inflammatory disorders, including rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), psoriatic arthritis (PsA), and systemic sclerosis (SSc). These conditions often result in chronic pain, disability, and reduced quality of life, with unpredictable disease courses that may lead to joint destruction, organ damage, or systemic complications. Biomarkers, defined as measurable indicators of biological processes or conditions, have the potential to transform clinical practice by improving disease diagnosis, monitoring, prognosis, and treatment decisions. While significant strides have been made in identifying and validating biomarkers in rheumatic diseases, challenges remain in their standardization, clinical utility, and integration into routine practice. This review provides an overview of the current state of biomarkers in rheumatic diseases, their roles in clinical settings, and the emerging advancements in the field
Churg-Strauss syndrome and Well's syndrome: coincidence or pathogenetic association ? A new case report
No full textA case of association of Churg-Strauss syndrome and Well's syndrome is reported. A possible common pathogenetic backgroud is briefly discussed
La iperostosi scheletrica idiopatica diffusa (DISH) [Diffuse Idipathic Skeletal Hyperostosis (DISH)]
No full textLa iperostosi scheletrica idiopatica diffusa (D.I.S.H.) è una patologia dismetabolica e degenerativa sistemica, caratterizzata da una metaplasia ossificante delle entesi. Le manifestazioni periferiche della malattia ricoprono grande importanza diagnostica in quanto precedono quelle assiali di molti anni. La D.I.S.H. deve essere tenuta distinta dall’artrosi, con la quale peraltro spesso coesiste, per il differente organo bersaglio del processo patologico, che, nell’artrosi, è invece primariamente rappresentato dalla cartilagine articolare.
La sintomatologia è variabile da forme asintomatiche o paucisintomatiche fino a quadri severi di compressione midollare. Risulta, infine, sempre opportuna la ricerca delle alterazioni metaboliche e delle patologie ad esse correlate.Diffuse idiopathic skeletal hyperostosis (D.I.S.H.) is a common disorder of unknown aetiology characterized by exuberant hyperostosis of the antero-lateral aspect of the spinal column, that sometimes leads to bone ankilosis, and by ossification of extra-spinal entheses. This condition is often associated with the metabolic derangement of type 2 diabetes. Primary hypertension, its cardiovascular aftereffects and lithiasis are also often present in these patients. D.I.S.H. has to be distinguished from osteoarthritis, although they often coexist in the same patient. The mean difference lies in the anatomical target of the pathological process, that is represented by articular cartilage in osteoarthritis and by entheses in diffuse idiopathic skeletal hyperostosis. The enthesopathy leads to the ossification of the anterior longitudinal ligament of the spine and causes the formation of flowing osteophytes, while intervertebral disc space is quite preserved in early phases of the disease. Symptoms of spine involvement are not typical of the disease and consist of pain and stiffness, usually worsened by inaction and damp. It has also been described the ossification of posterior longitudinal ligament which can lead to medullary canal stenosis. Appendicular skeleton is symmetrically involved in early phases of the disease, the most distinctive affected sites being feet, olecranus and patella. Hip involvement is also frequent and may lead to severe disability and represents an important cause of invalidity. The purpose of the present review is to remark on aetiopathogenetic and clinical aspects of diffuse idiopathic skeletal hyperostosis
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