Paraneoplastic myopathy in pancreatic cancer: a case report and literature review.

Dermatomyositis (DM) and polymyositis (PM) are both immune-mediated inflammatory myopathies known to occur in paraneoplastic syndromes associated with a new diagnosis of malignancy, most commonly breast, ovarian, lung, pancreatic, stomach, colorectal, and Non-Hodgkin's lymphoma1 in DM and breast, lung, bladder cancer, and Non-Hodgkin's lymphoma in PM. 2,3,4 While inflammatory markers such as creatine kinase (CK) may be elevated with either DM or PM, marked elevation is rare. Herein, we report a case of newly diagnosed pancreatic cancer presenting with inflammatory myopathy and marked CK elevation. We review the frequency of PM as a paraneoplastic syndrome, the association with marked CK elevation, and the association with pancreatic cancer.

IgG4 sclerosing disease of the esophagus: a case-based review.

IgG4-related disease (IgG4-RD) is an inflammatory and fibrosing disease which causes tumor-like swelling of organs and commonly mimics symptoms of malignancy. It has been increasing in prevalence in the last decade, but esophageal involvement remains rare. IgG4-RD was first known to involve certain organs, such as the pancreas. It has, since, been described as a systemic disease process. IgG4-RD should be considered in patients presenting with dysphagia. Initiation of appropriate treatment with corticosteroids can avoid unnecessary procedures and improve outcomes. The aim of this review is to discuss 17 cases of IgG4-RD of the esophagus. Literature review was conducted using NCBI database (PMC and PubMed filters) using the keywords "IgG4 disease," "sclerosing," "esophagus" and "gastrointestinal." The search was narrowed to include cases describing IgG4 disease of the esophagus using the same filters. Literature review identified 16 documented cases of IgG4-RD involving the esophagus. Upon literature review, it remains clear that it is extremely rare for IgG4-RD to affect the esophagus. Sixteen cases have been reported. We present a 17th case and discuss the implications of IgG4-RD. It is important to keep a broad differential diagnosis that includes IgG4-RD for patients presenting with dysphagia, especially when symptoms are refractory.

Aortitis caused by antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis: a case-based review.

Antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is a systemic necrotizing small vessel vasculitis primarily affecting elderly patients. Neutrophil apoptosis and release of pro-inflammatory mediators promote small vessel inflammation and hence multi-organ disease. It rarely affects larger vessels with extremely rare aortic involvement. Diagnosis is made based on clinical presentation, tissue biopsy of affected organ, as well as immunofluorescence and ELISA assays for ANCA. Management includes immunosuppression (e.g., glucocorticoids, cyclophosphamide and rituximab) and supportive therapy. We present a rare case of a younger patient with AAV involving the aorta. The patient's diagnosis was supported by clinical presentation, systemic organ involvement, strongly positive c-ANCA, and skin as well as aortic tissue biopsy results. After failing multiple immunosuppressants, he responded well to rituximab with improved symptoms, inflammatory markers, and imaging findings. Based on our literature review, we were only able to find ten cases of ANCA-related vasculitis involving the aorta. This is the first reported case of successful treatment of AAV-related aortitis using rituximab. Our case report and literature review provide insight into treatment of severe cases of AAV with aortic involvement.

Closed trauma, Mycoplasma hominis osteomyelitis, and the elusive diagnosis of Good's syndrome.

Mycoplasma hominis septic arthritis has a well-established association with hypogammaglobulinaemia, but is rarely seen in immunocompetent hosts. An association also exists with a closed trauma and a predisposition to M hominis bone infections. In this clinical case report, a patient with M hominis osteomyelitis following a closed trauma was diagnosed using 16S ribosomal studies, and led to the diagnosis of a severe underlying immunodeficiency syndrome known as Good's syndrome.

Update: biomarkers for idiopathic inflammatory myopathies.

PURPOSE OF REVIEW: Establishing diagnoses and distinguishing active disease from chronic injury remain significant clinical challenges in idiopathic inflammatory myopathies (IIM). Recent 'discovery' approaches utilizing novel genomic and proteomic techniques have revealed candidate molecular biomarkers to augment clinical and classical histological data. RECENT FINDINGS: Whole blood and serum Type 1 interferons (IFN-1) and IFN-1 inducible genes are gaining traction as disease biomarkers in IIM. IFNß is emerging as a disease activity marker specifically for dermatomyositis. Recently, molecules associated with innate immune-cell function, including TLR-3, high mobility group box (HMGB)-1, B7 Homolog 1, S100A4, and resistin have been detected in tissues of dermatomyositis patients. Serum Interleukin-17 (IL-17) and IL-23 correlate with active disease in early IIM. Antibodies recognizing the Survival Motor Neuron complex have been newly identified in a subset of patients with polymyositis. Protein aggregates are potential disease activity sensors for inclusion body myositis. Skin and lung harbor potential biomarkers for IIM. SUMMARY: Recent advances in understanding the pathogenesis of IIM have led to discovery of molecules that are candidate biomarkers of disease activity. Type 1 interferon and myeloid-cell signatures are leading candidate markers for use in IIM activity monitoring.