RESUMO
Sjögren's syndrome (SS) is a chronic autoimmune disease characterized by lymphocytic infiltration of exocrine glands and B cell hyperreactivity. Lacrimal and salivary glands are the most commonly involved causing keratoconjunctivitis sicca and xerostomia. A wide variety of other glandular and extraglandular manifestations can occur in SS. Lymphocytic mastitis is a rare presentation of several conditions including diabetes mellitus and autoimmune disorders. We report a case of a 43-year-old woman with a four-year history of arthralgias and positive antinuclear antibodies who developed a right painless breast mass. Biopsy revealed lymphocytic mastitis with predominant B cells. One year later she developed severe constitutional symptoms, sicca symptoms, lymphadenopathy, anemia, and interstitial lung disease. Serologies and minor salivary gland were consistent with the diagnosis of SS. This case further supports the association of lymphocytic mastitis with autoimmune diseases and demonstrates that it can even precede the clinical diagnosis of these entities.
Assuntos
Mastite/patologia , Síndrome de Sjogren-Larsson/diagnóstico , Adulto , Feminino , Humanos , Linfócitos , Mastite/etiologia , Síndrome de Sjogren-Larsson/complicações , Fatores de TempoRESUMO
A woman with Takayasu arteritis is reported who presented with constitutional symptoms and persistent thrombocytosis documented since 3 years before the diagnosis. Disease-specific symptoms such as arm claudication, transient loss of vision, and self-remitting eye ptosis present at the time were apparently missed, because she is a non-English-speaking Hispanic woman whose history was obtained through an interpreter. Extensive workup done at the time failed to reach a definite etiology. A computed tomography scan of the chest done because of midthoracic back pain and an elevated erythrocyte sedimentation rate showed circumferential wall thickening and mild surrounding edema throughout the thoracic and abdominal aorta and both carotid arteries consistent with Takayasu arteritis. Prednisone at a dosage of 1 mg/kg twice a day decreased the platelet count within 45 days of its initiation. Takayasu arteritis should be considered in the differential diagnosis of unexplained thrombocytosis, particularly in young women.
Assuntos
Arterite de Takayasu/diagnóstico , Trombocitose/etiologia , Adulto , Sedimentação Sanguínea , Feminino , Glucocorticoides/uso terapêutico , Humanos , Prednisona/uso terapêutico , Radiografia Torácica , Arterite de Takayasu/tratamento farmacológico , Trombocitose/tratamento farmacológicoRESUMO
OBJECTIVE: To characterize an IgA deficient population in terms of the incidence of IgG subclass and mannose-binding lectin (MBL) deficiencies and the type and severity of infections and other associated disorders. BACKGROUND: Selective IgA deficiency is probably the commonest of the primary immunodeficiency disorders and although it may lead to an increased risk for respiratory and gastrointestinal infections and associated to various autoimmune diseases, it may also be asymptomatic. Several studies have suggested the need of a concomitant defect in order for manifestation of its symptoms. METHODS: A total of 27 patients fulfilling the diagnostic criteria of selective IgA deficiency were evaluated for IgG subclass and MBL deficiencies after a thorough medical history, physical examination and pertinent evaluation for concomitant medical conditions. RESULTS: The overall incidence of IgG subclass deficiency found in the IgA deficient group was 18.5%. MBL deficiency was found to be 3.7%. These frequencies may have been influenced by the age group evaluated and the size of the population studied. Severe infections were more common in patients with combined deficiencies, either IgA and any of the IgG subclasses or IgA and MBL deficiency. Atopy was widely represented in the patients studied. CONCLUSIONS: The observed relationship between combined deficiencies Ig A, IgG subclasses and MBL and the increased representation of severe infections needs to be corroborated in a larger sample of patients with an inclusion of pediatric patients.