Latent autoimmune diabetes of adults (LADA) is a type of autoimmune diabetes that begins in adulthood (usually after the age of 35 years); its main feature is the presence of diabetes-associated autoantibodies (most often autoantibody against glutamic acid decarboxylase), which leads to progressive destruction of the islets of Langerhans. This is a heterogeneous condition that presents with clinical and laboratory manifestations common to type 1 diabetes and type 2 diabetes.We report a case of a 71-year-old man diagnosed with type 2 diabetes two years ago, poorly controlled with oral antidiabetic therapy, and worsening in the third year. He had a positive family history of type 2 diabetes in two second-degree relatives (nephews). No pathologic findings at the physical examination were found.His body mass index was 23 kg/m 2 and glycated hemoglobin was 10.6%. Laboratory workup revealed low basal C-peptide (<0.1 ng/mL) and positive glutamic acid decarboxylase antibodies, and the LADA diagnosis was confirmed. This case highlights the importance of being aware of this disease, especially in patients previously diagnosed with type 2 diabetes who remain uncontrolled with diet and oral hypoglycemic agents. LADA is often confused with type 2 diabetes, and therefore, the management is frequently inadequate. An early diagnosis and treatment are crucial to delaying disease progression.

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Younger patients and early referrals had better outcomes. Endothelin receptor antagonist toxicity should be monitored, particularly in patients previously exposed to hepatotoxic drugs.

SUMMARY The authors report a case of a 69-year-old man with idiopathic leukocytoclastic cutaneous vasculitis. For three years, the lesions recurred with progressive worsening and were associated with systemic manifestations of low-grade fever, weight loss and raised inflammatory markers. The patient latter presented a 6th cranial nerve involvement, raising the concern of a possible systemic vasculitis, which was latter evidenced by the development of deep vein thrombosis and angina pectoris. The treatment of the patient witch based on the decreasing of inflammatory activity, by using effective immunosuppressive therapy, with lower toxicity is more important than identifying the type of the vasculitis. This case illustrates the importance of awareness for the systemic involvement that can occur in up to 50% of patients with leukocytoclastic cutaneous vasculitis.

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Background In systemic sclerosis (SSc), digital ulcers (DU) are debilitating and sometimes recurrent complications. Treatment of DU is not dissociable from that of Raynaud’s phenomenon (RF), both express the same microvascular disfunction at different stages. Objectives Our goal is clinical characterization of a population of SSc patients with DU, its treatment, complications and outcome. Methods Retrospective analysis of 46 consecutive SSc patients between 1999-2012, meeting ACR or Le Roy classification criteria, from our department in a general hospital serving approximately 200.000 inhabitants. Clinical data were obtained from patient files. Results DU were detected in 10 patients: three males and 7 females, with a mean age of 60,2 years (range 48-76 years). Nine patients had limited type SSc (two had digital calcinosis) and one had diffuse type scleroderma. Anticentromere autoantibodies (ACA) were positive in 8 patients, anti-topoisomerase antibodies were present in one patient. Antiphospholipid antibodies were determined in six patients and were positive in one. All 10 patients were treated with calcium channel blockers (CCB) for RF. Healing of DU was made with intravenous prostanoid in 9 patients, four patients required repeated treatment with prostanoid for DU healing, one of these has a very slowly healing ulcer and positive antiphospholipid autoantibodies (APLA). After DU healing, there was persistent RF in three patients, two of them required repeated prostanoid infusions. Nine patients were later treated with bosentan, one of which experienced recurrence of DU. Alteration of liver function tests (LFT) was present in three patients: persistent in one patient with a history of alcohol intake, so bosentam was stopped; two were previously exposed to methotrexate, and LFT improved after folinic acid theraphy. Antiaggregant therapy was prescribed in nine patients and oral anticoagulants in two patients. Two patients had digital loss and two other patients healed with scars. Patients with digital loss were late presenters. The only patient that required hospitalization presented at diagnosis with pulmonary arterial hypertension (PAH), DU and digital loss; PAH normalized after treatment with bosentan. Two patients are dead: one died with massive digestive bleeding; the other died of liver failure, he had a history of alcohol intake. One patient is lost to follow-up. Three patients are still professionally active. Conclusions DU are serious manifestations of vasculopathy, a sign of target-organ damage; they are associated with increased morbidity (hand disability, reduced quality of life) and mortality. Treatment of DU comprises detection of complications, promotion of healing and preventing recurrence. Patients with SSc and DU have a worse prognosis, but there is sufficient evidence to support recommendations on standard of care and the most effective drugs available. Intravenous prostanoids and CCB have the highest level of evidence. Bosentan is recommended to prevent recurrence of DU, improving outcome. ...

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IntroductionLimbic encephalitis is an inflammatory disorder, commonly characterized by psychiatric features and cognitive impairment. It used to be considered a rare disease, usually paraneoplastic, with poor prognosis. Recent findings of membrane-surface directed antibodies, including anti-VGKC (Voltage-Gated Potassium Channels) antibodies, showed a substantial proportion of cases with no association with any malignancy and better prognosis.Case presentationA 56-year-old woman was admitted to the psychiatric ward in Nov/2007, with an acute onset of psychomotor retardation and agitation periods, apathy and nihilistic symptoms. She improved very slowly with lorazepam, clomipramine and quetiapine.In Jun/2014, she was admitted again to the psychiatric ward with acute onset of intense headache, confusion, insomnia, agitation and prejudice ideas. There was a clinical deterioration with mnesic deficits, catatonic stupor, autonomic dysfunction and seizures. Complementary exams revealed: ESR>120mm; hyponatraemia; acelular CSF with slightly elevated protein; EEG with slow base activity; Brain MRI with diffuse cortical atrophy and multiples subcortical hyperintensities of frontoparietal regions on FLAIR and T2 sequences; and positive anti-VGKC antibodies. Initially there was a poor transitory improvement with high-dose steroids and intravenous immunoglobulins. After 5 months, she did plasmapheresis with relevant clinical improvement.Discussion and ConclusionsAbout 80% of anti-VGKC encephalitis patients have clinical improvement following prompt treatment. In this case, there was not as good improvement as initially expected probably because of the prolonged duration of disease prior to effective treatment. Given the good prognostic outcome with prompt initiation of immunotherapy, early recognition and diagnosis of this rare neuropsychiatric syndrome is crucial.