"Puffy hand syndrome".

Intravenous drug addiction is responsible for many complications, especially cutaneous and infectious. There is a syndrome, rarely observed in rheumatology, resulting in "puffy hands": the puffy hand syndrome. We report two cases of this condition from our rheumatologic consultation. Our two patients had intravenous drug addiction. They presented with an edema of the hands, bilateral, painless, no pitting, occurring in one of our patient during heroin intoxication, and in the other 2 years after stopping injections. In our two patients, additional investigations (biological, radiological, ultrasound) were unremarkable, which helped us, in the context, to put the diagnosis of puffy hand syndrome. The pathophysiology, still unclear, is based in part on a lymphatic toxicity of drugs and their excipients. There is no etiological treatment but elastic compression by night has improved edema of the hands in one of our patients.

Dysregulated serum IL-23 and SIRT1 activity in peripheral blood mononuclear cells of patients with rheumatoid arthritis.

Sirtuin 1 (Sirt1) is a class III histone deacetylase (HDAC) that modulates gene expression and is involved in the regulation of proinflammatory cytokines. Interleukin-23 (IL-23) is produced by activated macrophages and dendritic cells and could fuel the progression of rheumatoid arthritis (RA). The goal of our study was to evaluate serum IL-23 levels and both Sirt1 activity and expression in peripheral blood mononuclear cells (PBMCs) in patients with RA compared to healthy controls (HC) and to determine the relationship between Sirt1 activity/expression and IL-23 levels. We assessed apoptosis in PBMCs of RA patients and its association with Sirt1 expression and serum IL-23. Serum IL-23 levels were increased in RA patients in comparison with controls. We found a positive correlation between the levels of serum IL-23 and serum IL-6 in RA patients. Decreased cytoplasmic Sirt1 activity was observed in RA patients with severe disease compared to HC. The expression of Sirt1 protein was significantly decreased in PBMCs of RA patients compared to HC using western blotting. Serum IL-23 levels correlated positively with the cytoplasmic Sirt1 activity in RA patients. Apoptosis rate of PBMCs isolated from RA patients was increased compared to HC and correlated negatively with the expression of Sirt1 protein and serum IL-23 levels. Levels of serum IL-23 and Sirt1 activity and expression were disturbed in RA parallel to increased PBMC apoptosis. Our findings might provide the rationale for the development of new therapeutic approaches in RA.

Myelofibrosis-related arthritis successfully treated with hydroxyurea.

A 62-year-old woman suffering from one-year lasting, nonerosive peripheral arthritides with general health impairment and high acute-phase reactant levels was admitted to rheumatology department. The patient had suffered from chronic polyarthralgia and a thrombocytosis had been discovered 9 years before, with a recent increase in platelet count. All immunological blood tests were negative. Corticosteroid and methotrexate treatments improved pain, swollen joint count, and systemic inflammation. However, joints remained stiff and painful with two swollen wrists and persistent thrombocytosis. An iliac bone marrow biopsy was performed, showing primary myelofibrosis. Hydroxyurea treatment (500 mg per day) allowed to achieve complete and prolonged clinical and biological remission. After 6 months, a new disease flare occurred. The patient reached remission again after hydroxyurea dose increased to 1500 mg per day. This supports the hypothesis of idiopathic myelofibrosis-associated seronegative polyarthritis. This is the first reported case in which haemopathy-targeted treatment using hydroxyurea induced arthritis remission.

When gout involves the spine: five patients including two inaugural cases.

UNLABELLED: Spinal involvement is uncommon during gout and may raise diagnostic challenges. We describe five cases seen at a single center. METHODS: We retrospectively reviewed the medical charts of the five patients with spinal gout seen over a 3-year period. RESULTS: There were four men and one woman with an age range of 52 to 87 years. One patient presented with acute neck pain and visualization by imaging studies of a discovertebral tophus, another had febrile arthritis of a lumbar facet joint, and a third presented with a synovial cyst in a lumbar facet joint. The remaining two patients had acute febrile discitis confirmed by magnetic resonance imaging, at the cervical spine and lumbar spine, respectively. Laboratory tests showed systemic inflammation in four patients and marked serum uric acid elevation in two patients. Only three patients reported a previous history of peripheral acute gout attacks. Specimens of the spinal lesions were obtained in three patients and consistently showed monosodium urate crystals with tissue inflammation or a tophus. The outcome was rapidly favorable, either with colchicine therapy alone in four patients or after surgical resection of a facet joint cyst (during surgery to stabilize the lumbar spine) in the remaining patient. The patient with neck pain due to a tophus experienced nerve root pain at the acute phase. No other neurological manifestations were recorded. CONCLUSION: These case reports illustrate the diagnostic challenges raised by spinal involvement due to gout. The spinal lesions can be inaugural, as seen in two of our five patients.