Primary Sjögren’s syndrome (SS) is a chronic, systemic autoimmune disease, most commonly presenting with sicca symptoms—xerostomia and xerophthalmia. Up to one-half of affected individuals also develop extra-glandular involvement. Early diagnosis is important to prevent further complications. This paper describes an atypical case of SS in a 21-year-old woman who presented first with neuropsychiatric symptoms and fever. She was diagnosed and treated for a presumed psychotic episode. However, because she remained febrile, a thorough investigation resulted in the challenging diagnosis of SS.LEARNING POINTSPrimary Sjögren’s syndrome (SS) may be an under-recognized cause of central nervous system and neuropsychiatric involvement.Neuropsychiatric manifestations of primary SS can be variable.Patients may be treated incorrectly if the diagnosis is not rapidly established, so a clinical history and thorough examination are very important.

Mixed connective tissue disease (MCDT) is a rare condition characterised by the presence of high titres of anti-U1 ribonucleoprotein antibodies and selected clinical features of systemic lupus erythematosus, systemic sclerosis and polymyositis/dermatomyositis. Early symptoms are non-specific, including easy fatigability, myalgia, arthralgia and Raynaud's phenomenon. Some reports emphasised the favourable outcome and excellent response to glucocorticoids, but there are contradictory studies reporting worse prognosis. Also, a subset of patients evolve into a clinical picture more consistent with a major diffuse connective tissue disease. We present the case of a 50-year-old black woman whose inaugural presentation of MCDT was oropharyngeal dysphagia, symmetrical proximal muscle weakness, tongue atrophy and skin sclerosis. High-dose corticosteroids and methotrexate were given with little improvement, maintaining disabling dysphagia leading to a percutaneous endoscopic gastrostomy tube placement. She was then started on intravenous immunoglobulin with progressive remission of symptoms.

Eales’ disease is a peripheral occlusive retinal phlebitis, with an unclear pathogenesis. The classic association with hypersensitivity to Mycobacterium tuberculosis protein infers that immunologic disturbance may be involved. Here, we described three cases of Eales’ disease. All patients are Caucasian men aged 27-58 years and presented with vitreous hemorrhage and/or peripheral venous vasculitis. Tuberculin skin sensitive test (Mantoux screening test) and interferon-gamma release assay (IGRA) were positive in all patients. Therapeutic approach included antituberculosis therapy and systemic steroids, associated or not to immunosuppressive therapy, and retinal scatter photocoagulation in all cases. Antivascular endothelial grow factor (VEGF) intravitreal injections were also required in two cases. Since various retinal diseases can resemble this presentation, Eales’ disease is considered a diagnosis of exclusion. Early diagnosis and appropriate therapeutic approach are both essential to accomplish disease control and reduce ophthalmologic complications.

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