Deficiencies of the early complement components of the classical pathway (CP) are well-documented in association with systemic lupus erythematosus (SLE) or SLE-like syndromes and severe pyogenic infections. Among these, complete C1s deficiency has been reported in nine cases so far. Here, we describe a 34-year-old male patient who presented with severe, recurrent infections since childhood, including meningitides with pneumococci and meningococci, erysipelas, subcutaneous abscess, and recurrent infections of the upper airways.
View Article and Find Full Text PDFBackground: Over the last decades, the prevalence of coeliac disease (CD), an autoimmune disorder, rose to 1-2%. Whether patients with CD have higher risk of developing other autoimmune disorders such as type 1 diabetes, Hashimoto thyroiditis, or Graves` disease remains unclear.
Aim: The aim of this study was to determine the prevalence of biomarkers of beta cell and thyroid autoimmunity in children with CD.
Background: Free light chains, type kappa (FLC-K), in cerebrospinal fluid (CSF) were compared to oligoclonal IgG in many studies for sensitive detection of immune reactions in brain. The missing consensus about CSF data interpretation prevents reliable conclusions. This can be overcome by a theory-based hyperbolic reference range in CSF/serum quotient diagrams.
View Article and Find Full Text PDFBackground The quantification of serum IgG4 is commonly performed during the diagnostic workup of IgG4-related diseases (IgG4-RD). According to recent literature, IgG4 values above 1.35 g/L are characteristic of IgG4-RD and support its diagnosis at initial presentation.
View Article and Find Full Text PDFBackground: Early diagnosis and relapse prediction in Graves' disease influences treatment. We assessed the abilities of four TSH-receptor antibody tests [TRAb] and one cyclic adenosine monophosphate bioassay to predict relapse of Graves' disease.
Methods: Observational study investigating patients presenting with Graves' disease at a Swiss hospital endocrine referral center or an endocrine outpatient clinic.
Objective: Adrenocorticotrophic hormone (ACTH) stimulation testing is the current standard for assessing primary and secondary adrenal insufficiency (AI). We aimed to investigate the value of basal cortisol level for prediction of AI.
Methods: We retrospectively analyzed 804 consecutive patients who had high-dose (250 μg, HDT) or low-dose (1 μg, LDT) ACTH testing as part of their diagnostic work-up.
Context: First-line treatment in Graves' disease is often done with antithyroid agents (ATD), but relapse rates remain high making definite treatment necessary. Predictors for relapse risk help guiding initial treatment decisions.
Objective: We aimed to externally validate the prognostic accuracy of the recently proposed Graves' Recurrent Events After Therapy (GREAT) score to predict relapse risk in Graves' disease.