Neuro-Behçet's disease, its mimickers and anti-TNF therapy: a case-based review.

When the central nervous system is the primary affected site in an initial attack of Behçet's disease (BD), the differential diagnosis is particularly challenging. Because the specificity of immunobiologic therapy is growing, the specific diagnosis may impact the chosen therapy. For instance, anti-tumour necrosis factor agents are efficacious in BD but may be harmful in multiple sclerosis or systemic lupus erythematosus. We present two cases with similar neurological features but different diagnosis (BD and systemic lupus erythematosus) as a starting point to review diagnostic and therapeutic approaches for neuro-BD and its differential diagnoses.

Infliximab treatment in pachydermoperiostosis: a rare disease without an effective therapeutic option.

Hypertrophic osteoarthropathy (HOA) is characterized by periostitis of tubular bones, thickened skin, and digital clubbing. Its pathogenesis is unknown but an inflammatory factor and increased bone remodeling have been implicated. It is a very rare disease, usually diagnosed late with few therapeutic options. Bone and joint pains are secondary to periostitis and are usually difficult to control. Tumor necrosis factor-alpha is a cytokine that induces other inflammatory cytokine production, has an osteoclastogenic effect in different rheumatic diseases and probably also has an important role in periostitis and the systemic inflammatory manifestations in HOA. We describe the case of a patient with the primary form of HOA, who had refractory bone pain and arthritis that responded partially to infliximab treatment.

Single measurements of C-reactive protein and disease activity scores are not predictors of carotid atherosclerosis in rheumatoid arthritis patients.

BACKGROUND: Although inflammation has a defined role in the pathogenesis of atherosclerosis, the link between rheumatoid arthritis (RA) parameters of disease activity and atherosclerotic findings are not defined. OBJECTIVE: To investigate the association between subclinical carotid atherosclerosis and clinical/laboratorial parameters of RA systemic inflammatory activity. METHODS: Seventy-one RA patients were consecutively selected and compared to 53 healthy controls. Smoking, diabetes and hypertension were excluded, as well as the use of statins or fibrates. B-mode carotid ultrasound was performed in all subjects. CRP, ESR and fibrinogen were determined in both groups. Clinical assessment of RA activity included DAS 28 and SDAI. Correlation between plaques and intima-media thickness (IMT) of common carotid arteries and inflammatory parameters was evaluated. RESULTS: Carotid plaques were more prevalent in RA patients than in controls (14.1% vs. 1.9 %, p=0.02) and marginally increased IMT was observed (0.72 +/- 0.17 vs. 0.67 +/- 0.15 mm, p=0.07). RA patients with plaques had older age (p=0.001) and increased IMT (p<0.001), but low SDAI (p=0.025) compared to those without plaques. RA patients with plaques had also longer disease duration, although this difference did not reach statistical significance (p=0.06). No significant correlations were found between IMT and ESR (p=0.80), CRP (p=0.75), fibrinogen (p=0.94), HAQ (p=0.89) and DAS 28 (p=0.13). CONCLUSIONS: Carotid atherosclerosis is more frequently detected in RA but its prevalence was not correlated with isolated inflammatory markers measurement or noncumulative activity scores. These findings reinforce the need to evaluate subclinical atherosclerosis in RA patients, and to find predictors of atherosclerotic lesions.

Pulmonary amyloidosis in Sjögren's syndrome: a rare diagnosis for nodular lung lesions.

Lung involvement in Sjögren's syndrome (SS) can affect trachea, bronchus, small airways, pleura and may cause interstitial lung injury. It may also be associated with malignancies, particularly non-Hodgkin's lymphoma, which is a well-recognized complication of this disease. Here we describe the occurrence of localized amyloidosis presenting as pulmonary nodules in a patient with newly diagnosed SS. We highlight this rare occurrence as a diagnostic possibility that should be considered in the evaluation of pulmonary involvement in this disease.

Artropatia ocronótica: manifestaçoes cardiovasculares e associaçao com artrite reumatóide: relato de dois casos / Ochronotic arthropathy: cardiovascular manifestations and association with rheumatoid arthritis: report of two cases

Os autores relatam dois casos de ocronose alcaptonúrica apresentando a artropatia característica, com comprometimento periférico e axial. Uma paciente tinha também dados clínicos e radiológicos compatíveis com artrite reumatóide. A outra desenvolveu estenose aórtica calcificada, complicaçao cardíaca relacionada à doença. Enfatiza-se a importância de criteriosa avaliaçao do quadro articular e do sistema cardiovascular em um paciente portador de ocronose. Os conceitos fundamentais acerca desta rara doença sao brevemente revistos