Monoclonal gammopathy of clinical significance: what the rheumatologist needs to know.

Monoclonal proteins can provide important information on the diagnosis of several non-malignant systemic inflammatory disorders. At low concentration, they most commonly represent monoclonal gammopathy of undetermined significance (MGUS), whereas high concentrations often signify plasma cell myeloma or B-cell lymphoma. However, several rare inflammatory conditions associated with variable concentrations of monoclonal proteins, systemic symptoms, and organ dysfunction also exist.

IgG4-related disease and lymphocyte-variant hypereosinophilic syndrome: A comparative case series.

Objective: To compare the clinical and laboratory features of IgG4-related disease (IgG4-RD) and lymphocyte-variant hypereosinophilic syndrome (L-HES), two rare diseases that often present with lymphadenopathy, gastrointestinal symptoms, eosinophilia, and elevated immunoglobulins/IgE.

Method: Comparative case series of 31 patients with IgG4-RD and 13 patients with L-HES.

Results: Peripheral blood eosinophilia was present in eight of 31 patients with IgG4-RD compared to 13 of 13 patients with L-HES (median eosinophils 0.

Recognizing IgG4-related tubulointerstitial nephritis.

Purpose Of The Review: Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory disorder affecting nearly all organs, including the kidney. Tubulointerstitial nephritis (IgG4-TIN) is the most common form of IgG4-related kidney disease (IgG4-RKD) and is the focus of this concise review.

Objective: The study aims to describe when IgG4-TIN should be suspected and to summarize the diagnosis, treatment, and natural history of the disease.

Hemophagocytic syndromes (HPSs) including hemophagocytic lymphohistiocytosis (HLH) in adults: A systematic scoping review.

Most knowledge of hemophagocytic syndromes (HPSs) including hemophagocytic lymphohistiocytosis (HLH) is derived from pediatric studies; literature on adult HPS/HLH predominantly consists of small retrospective studies with clinical and methodological heterogeneity. The aims of this systematic scoping review were to provide an overview of existing literature on adult HPS/HLH, describe current practices in diagnosis and treatment, and propose priorities for future research. Articles from Ovid Medline, Embase and Pubmed (1975-2015) describing 10 or more unique adults (age>15years) with HPS/HLH were included.

Polyclonal hyperviscosity syndrome in IgG4-related disease and associated conditions.

Polyclonal hyperviscosity syndrome (HVS) is rare and has been reported in various disorders of immune dysregulation and lymphoid hyperplasia. IgG4-Related Disease (IgG4-RD) is an emerging disorder often associated with exuberant hypergammaglobulinemia, and this review of seven cases establishes IgG4-RD as an important cause of polyclonal HVS.

Abnormalities of the lymphocyte subsets and their immunophenotype, and their prognostic significance in adult patients with hemophagocytic lymphohistiocytosis.

Hemophagocytic lymphohistiocytosis (HLH) is a final common pathway resulting from diverse immune processes. Most of the current understanding of HLH is based on studies involving pediatric patients (pHLH). Adult HLH (aHLH) is distinct from pHLH, with less frequent genetic basis, higher frequency of chemo-resistance and a higher mortality.

Residents' views of the role of classroom-based learning in graduate medical education through the lens of academic half days.

Purpose: To examine the role of classroom-based learning in graduate medical education through the lens of academic half days (AHDs) by exploring residents' perceptions of AHDs' purpose and relevance and the effectiveness of teaching and learning in AHDs.

Method: The authors invited a total of 186 residents in three programs (internal medicine, orthopedic surgery, and hematology) at the University of British Columbia Faculty of Medicine to participate in semistructured focus groups from October 2010 to February 2011. Verbatim transcripts of the interviews underwent inductive analysis.

IgG4-related disease with hypergammaglobulinemic hyperviscosity and retinopathy.

Immunoglobulin G4-related disease (IgG4-RD) is a recently described entity with protean manifestations. We describe a novel case of IgG4-RD with hypergammaglobulinemic hyperviscosity responsive to fludarabine and rituximab. A 33-year-old Asian man developed bilateral lacrimal gland and submandibular salivary gland swelling with cervical lymphadenopathy.