Dionicio Ángel Galarza-Delgado

Takayasu's arteritis and posterior reversible encephalopathy syndrome: a case-based review

Autoimmune vasculitides can have diverse neurological manifestations, including posterior reversible encephalopathy syndrome (PRES). Takayasus arteritis (TA) is an uncommon vasculitis rarely associated with PRES. Common clinical features of TA include hypertension, audible arterial bruits, absence of peripheral pulses, claudication of the extremities, reduced blood pressure in one or both arms, and angiographic abnormalities. PRES has been mostly associated with severe hypertension, endothelial injury, and conditions such as renal disease, immunosuppressive medication use, and rheumatologic diseases. Headaches, seizures, and altered mental status are the main clinical features as well as characteristic findings in magnetic resonance imaging. TA frequently presents with hypertension and is associated with endothelial injury, making this entity an ideal setting for the development of PRES. We report the case of a 17-year-old female who presented to the emergency department with severe hypertension, headache, and seizures. Magnetic resonance imaging findings were suggestive of PRES. She had absent pulses in the right upper extremity, abdominal bruits, and angiographic findings included subclavian and renal artery stenoses. The diagnosis of TA was made, and she responded well to treatment. We found ten additional cases of TA and PRES in the literature. All patients were females under the age of 40, had renovascular hypertension, and presented with headaches and seizures. Current literature relevant to this rare association is presented and discussed.

Clinical Features and Outcome of Mucormycosis

Mucormycosis (MCM) is a life-threatening infection that carries high mortality rates despite recent advances in its diagnosis and treatment. The objective was to report 14 cases of mucormycosis infection and review the relevant literature. We retrospectively analyzed the demographic and clinical data of 14 consecutive patients that presented with MCM in a tertiary-care teaching hospital in northern Mexico. The mean age of the patients was 39.9 (range 5–65). Nine of the patients were male. Ten patients had diabetes mellitus as the underlying disease, and 6 patients had a hematological malignancy (acute leukemia). Of the diabetic patients, 3 had chronic renal failure and 4 presented with diabetic ketoacidosis. All patients had rhinocerebral involvement. In-hospital mortality was 50%. All patients received medical therapy with polyene antifungals and 11 patients underwent surgical therapy. Survivors were significantly younger and less likely to have diabetes than nonsurvivors, and had higher levels of serum albumin on admission. The clinical outcome of patients with MCM is poor. Uncontrolled diabetes and age are negative prognostic factors.

Hormona anti-mülleriana en mujeres en edad reproductiva con lupus eritematoso sistémico

UNLABELLED Systemic lupus erythematosus (SLE) is an inflammatory autoimmune systemic and chronic disease. Fertility in SLE patients is considered normal; factors that have been associated in these patients with ovarian failure are: disease activity, autoantibodies, and the use of cytotoxic agents. The anti-Müllerian hormone (AMH) is a marker that helps to determine the follicular reserve. OBJECTIVE Determinate the objective was to determine AMH levels in women of reproductive age with SLE. MATERIAL AND METHODS We included 65 women with SLE classified according to the 1997 ACR criteria, 18- to 40-years old. We obtained demographic, clinical, obstetric, and gynecological characteristics as well as serum levels of AMH. We performed a bivariate analysis among patients with low ovarian reserve and those with normal ovarian reserve. We also performed a correlation analysis between activity and damage index and between the cumulative cyclophosphamide dose and AMH levels. RESULTS We found a median of serum AMH in SLE patients of .61 ng/mL. The prevalence of low ovarian reserve in our study was 3.07%. We found a median MEX-SLEDAI score of 1 point and the median SLICC score was 2 points. Twenty-five patients (38.4%) had used cyclophosphamide and their cumulative average dose was 7.5 grams. CONCLUSIONS We found a median of AMH of .61 ng/mL in our population. The prevalence of low ovarian reserve in SLE patients was 3.07%. We did not find a correlation between AMH levels, the use of cyclophosphamide, and disease activity.

O volume plaquetário médio está reduzido em adultos com lúpus ativo

BACKGROUND Only a few biomarkers are available for assessing disease activity in systemic lupus erythematosus (SLE). Mean platelet volume (MPV) has been recently studied as an inflammatory biomarker. It is currently unclear whether MPV may also play a role as a biomarker of disease activity in adult patients with SLE. OBJECTIVE We investigated the association between MPV and disease activity in adult patients with SLE. METHODS In this retrospective study, we compared two groups of adult patients divided according to disease activity (36 per group). Subjects were age- and gender-matched. RESULTS MPV was significantly decreased with respect to those of inactive patients (7.16±1.39 vs. 8.16±1.50, p=0.005). At a cutoff level of 8.32 fL, MPV has a sensitivity of 86% and a specificity of 41% for the detection of disease activity. A modest positive correlation was found between MPV and albumin (r=0.407, p=0.001), which in turn is inversely associated with disease activity. CONCLUSIONS In summary, MPV is decreased in adult patients with active lupus disease, and positively correlated with albumin, another biomarker of disease activity. Prospective studies are needed to evaluate the prognostic value of this biomarker.

Posterior Reversible Encephalopathy Syndrome Due to Malignant Hypercalcemia: Physiopathological Considerations

CONTEXT Posterior reversible encephalopathy syndrome (PRES) is a neurological entity characterized by seizures, headache, and reversible subcortical vasogenic edema. It is associated with many etiologies, most often hypertension, chronic renal failure, and chemotherapy. Hypercalcemia is rarely associated with PRES. OBJECTIVE The aim of this study is to describe and discuss a case of PRES that developed in a patient with malignant hypercalcemia, with emphasis on the possible pathophysiological mechanisms involved. PATIENTS AND METHODS A 38-year-old woman presented with altered mental status. She had a 2-month history of lumbar pain of moderate intensity, weight loss, and gastrointestinal complaints, in addition to a mass in her left breast. Her corrected serum calcium was 14.5 mg/dL. She was normotensive, had no focalizing signs, and her cerebrospinal fluid was normal. Despite treatment, her neurological state did not resolve, and she developed severe headaches at day 4 of her admission. Brain magnetic resonance imaging showed a bilateral and symmetric hyperintensity in the occipital and parietal lobes on T2-weighted and fluid-attenuated inversion recovery imaging, a characteristic highly suggestive of PRES. After correction of hypercalcemia, her symptoms and imaging abnormalities resolved. CONCLUSIONS The development of PRES in the setting of severe hypercalcemia is extremely rare. Hypercalcemia could lead to PRES in the absence of hypertension by various mechanisms, including vasospasm, endothelial dysfunction, and an inflammatory state. A high index of suspicion is needed in this setting because hypercalcemia can lead to neurological symptomatology, and prompt diagnosis is essential for adequate treatment.

Mean platelet volume is decreased in adults with active lupus disease

BACKGROUND Only a few biomarkers are available for assessing disease activity in systemic lupus erythematosus (SLE). Mean platelet volume (MPV) has been recently studied as an inflammatory biomarker. It is currently unclear whether MPV may also play a role as a biomarker of disease activity in adult patients with SLE. OBJECTIVE We investigated the association between MPV and disease activity in adult patients with SLE. METHODS In this retrospective study, we compared two groups of adult patients divided according to disease activity (36 per group). Subjects were age- and gender-matched. RESULTS MPV was significantly decreased with respect to those of inactive patients (7.16±1.39 vs. 8.16±1.50, p=0.005). At a cutoff level of 8.32fL, MPV has a sensitivity of 86% and a specificity of 41% for the detection of disease activity. A modest positive correlation was found between MPV and albumin (r=0.407, p=0.001), which in turn is inversely associated with disease activity. CONCLUSIONS In summary, MPV is decreased in adult patients with active lupus disease, and positively correlated with albumin, another biomarker of disease activity. Prospective studies are needed to evaluate the prognostic value of this biomarker.

Isolated rheumatoid arthritis-associated cerebral vasculitis: A diagnostic challenge

Central nervous system involvement in rheumatoid arthritis is uncommon. In order of frequency, published cases have reported rheumatoid nodules, meningeal vasculitis, and cerebral vasculitis (CV). The frequency of vasculitic cerebral involvement in rheumatoid arthritis is unknown. However, it is known that it is more common in patients with seropositive, long-standing rheumatoid arthritis, and in those with concomitant systemic vasculitis. We report the case of a 47-year-old woman with an 11-year history of seropositive rheumatoid arthritis without any extra-articular manifestations, with the exception of secondary Sjogrens syndrome, presenting with mental status changes and tonic-clonic seizures as symptoms of isolated CV. Magnetic resonance imaging (T2) showed hyperintense white-matter lesions in the frontal and temporal lobes, as well as in the hippocampus and cerebellum. Transcranial Doppler ultrasound findings were consistent with CV. Other differential diagnoses were ruled out. The patient responded favorably to methylprednisolone and intravenous gammaglobulin therapy.

Wernicke Encephalopathy Presenting in a Patient with Severe Acute Pancreatitis

CONTEXT Acute pancreatitis can lead to prolonged fasting and malnutrition. Many metabolic changes, including thiamine deficiency, may lead to the well know pancreatic encephalopathy. In this condition however the thiamine deficiency is rarely suspected. CASE REPORT We report the case of a 17-year-old woman with severe acute pancreatitis who developed mental status changes and ophthalmoplegia. A magnetic resonance image showed hyperintensive signals in periventricular areas, medial thalamus, and mammillary bodies, findings consistent with the diagnosis of Wernicke encephalopathy. Thiamine treatment reversed neurological complications. CONCLUSION Wernicke encephalopathy secondary to thiamine deficiency should be considered as a possible cause of acute mental status changes in patients with acute pancreatitis and malnutrition. Prophylactic doses of thiamine could be considered in susceptible patients.

Carotid atherosclerosis in patients with rheumatoid arthritis and rheumatoid nodules

OBJECTIVE To determine whether an association exists between the presence of rheumatoid nodules and thickening of the intima-media and plaque of the carotid artery, which is evidence of atherosclerosis. MATERIALS AND METHODS Observational, cross-sectional study of 124 patients with rheumatoid arthritis from a University Hospital clinic from 2005 to 2006. We divided the patients into 2 groups, 62 with rheumatoid nodules and 62 without rheumatoid nodules, matched for age and sex. Medical history, erythrocyte sedimentation rate, anti-cyclic citrullinated peptide, rheumatoid factor, and a high resolution doppler ultrasound of the carotid arteries were performed. RESULTS Women comprised 89.5% of the patients. The prevalence of a carotid plaque was 57% in our population. The presence of a plaque was associated with age, arterial hypertension and abdominal circumference. Average intima-media thickness (IMT) in patients with a plaque was 0.085 cm (± 0.02). There was no correlation between laboratory parameters and thickening of the intima-media of the carotid artery. Subcutaneous nodules were present in 33 (47%) of the 70 patients with a carotid plaque and in 29 (54%) of patients without a carotid plaque (p=.471). CONCLUSIONS We did not find an association between rheumatoid nodules and the presence of a carotid plaque and thickening of the intima-media of the carotid in patients with rheumatoid arthritis.

Case of chromoblastomycosis with pulmonary involvement

Chromoblastomycosis is a slowly growing chronic cutaneous mycosis associated with a variety of cutaneous lesions. Extra‐dermal involvement is rare. A 58‐year‐old man was admitted to the hospital with nausea, vomiting, weakness and a history of weight loss. On inspection, he had a large verrucous mass in the sacral region, and two large subcutaneous nodules in the anterior thoracic wall. He claimed the lesions were several years old. Biopsy and histological studies were positive for chromoblastomycosis. Routine chest radiography showed hilar enlargement, and a chest computed tomography was ordered. Pulmonary nodules were evident, and endoscopically acquired samples were also positive for chromoblastomycosis. Extra‐dermal and systemic involvement in chromoblastomycosis is exceedingly rare and often associated with immunosuppression. There is only one other case of pulmonary chromoblastomycosis reported in the published work.