Rheumatoid arthritis is characterized by the presence of inflammatory synovitis and destruction of joint cartilage and bone. Tissue proteinases released by synovia, chondrocytes and pannus can cause cartilage destruction and cytokine-activated osteoclasts have been implicated in bone erosions. Rheumatoid arthritis synovial tissues produce a variety of cytokines and growth factors that induce monocyte differentiation to osteoclasts and their proliferation, activation and longer survival in tissues. More recently, a major role in bone erosion has been attributed to the receptor activator of nuclear factor kappa B ligand (RANKL) released by activated lymphocytes and osteoblasts. In fact, osteoclasts are markedly activated after RANKL binding to the cognate RANK expressed on the surface of these cells. RANKL expression can be upregulated by bone-resorbing factors such as glucocorticoids, vitamin D3, interleukin 1 (IL-1), IL-6, IL-11, IL-17, tumor necrosis factor-α, prostaglandin E 2 , or parathyroid hormone-related peptide. Supporting this idea, inhibition of RANKL by osteoprotegerin, a natural soluble RANKL receptor, prevents bone loss in experimental models. Tumor growth factor-ß released from bone during active bone resorption has been suggested as one feedback mechanism for upregulating osteoprotegerin and estrogen can increase its production on osteoblasts. Modulation of these systems provides the opportunity to inhibit bone loss and deformity in chronic arthritis.
Described in 1962, the opsoclonus-myoclonus-ataxia syndrome (OMAS) is a rare, neurologically debilitating disorder with distinct characteristics that may begin in childhood or adult life. Although many cases remain without etiological diagnosis, others are related to neoplasms and infectious diseases. We report a 41-year-old previously healthy male with an 8-day history of headache, vertigo, nausea, vomiting, and nystagmus. After a normal brain computed tomography and lymphocytic pleocytosis in cerebral spinal fluid (CSF), intravenous acyclovir therapy was initiated in the emergency room. On the third day of hospitalization, the diagnosis of OMAS was made based on the presence of chaotic and irregular eye movements, dysarthric speech, gait instability, generalized tremor, and myoclonic jerks. In the face of his neurological worsening, ampicillin followed by nonspecific immunotherapy (methylprednisolone and intravenous immunoglobulin) was prescribed, with mild clinical improvement. After a thorough laboratory workup, the definite diagnosis of neuroborreliosis was established and ceftriaxone (4 g/daily/3 wks) and doxycycline (200 mg/day/2 mo) was administered. Toward the end of the ceftriaxone regimen, the neurologic signs substantially improved. We believe this to be the first case description of OMAS as clinical presentation of Brazilian Lyme disease-like syndrome (Baggio-Yoshinari syndrome).
Chronic meningococcemia is a rare clinical presentation within the spectrum of infections due to Neisseria -en.jpg-en.jpgmeningitidis, which was first described in 1902. It is defined as a chronic and benign meningococcal bacteremia without meningeal signs or symptoms with at least one week’s duration, characterized by intermittent or continuous fever, polymorphic cutaneous rash, and migratory arthropathy. The incidence is believed to be around 1:200,000 inhabitants. It affects predominantly young people and adults, and it is equally distributed between genders. Diagnosis may be challenging in the early stages of the disease because of the difficulty in isolating Neisseria -en.jpg-en.jpgmeningitidis (it reaches 74% of positivity in advanced stages). Recently, the use of PCR for detecting Neisseria -en.jpg-en.jpgsp antigen in skin biopsies specimens has been considered for those culture-negative cases. The authors report a case of a 54-year-old female patient who sought medical attention for a five-day fever followed by arthralgia and skin lesions predominantly in the lower limbs. The patient progressed to a toxemic clinical status that improved after the administration of antibiotic therapy, which consisted of oxacillin and ceftriaxone. The diagnosis of chronic meningococcemia was performed after the isolation of Neisseria meningitidis in two different blood sample cultures. This is, to our knowledge, the first case of chronic meningococcemia described in Brazil (up to the writing of this report).
(FMUSP). Recebido em 14/4/2001. Aprovado, após revisão, em 13/4/2003. 1. Médico(a) residente da Disciplina de Reumatologia do Hospital das Clínicas da FMUSP. 2. Médico assistente da Disciplina de Reumatologia do Hospital das Clínicas da FMUSP.
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