Belimumab in the Treatment of Connective Tissue Disease-Associated Interstitial Lung Disease: Case Report and Literature Review.

Interstitial lung disease or ILD can be described as inflammation, fibrosis, or scarring of the lung's interstitial, resulting in dyspnea. ILD represents a group of heterogeneous parenchymal lung disorders with complex pathophysiology, differentiated by the clinical and radiological patterns. ILD is one of the most serious pulmonary complications associated with connective tissue diseases (CTDs), resulting in significant morbidity and mortality. Nonspecific interstitial pneumonia is the most common morphological and pathological pattern of ILD seen in CTDs. There are limitations in the therapeutic options resulting in significant morbidity. Certain biologic therapies are being evaluated for the various forms of ILD. The ILD, in this case, is associated with systemic lupus erythematosus (SLE) and scleroderma overlap that was effectively treated with belimumab, a recombinant monoclonal antibody against the B-cell activating factor (B-lymphocyte stimulator).

Prevalence of Axonal Sensory Neuropathy With IgM Binding to Trisulfated Heparin Disaccharide in Patients With Fibromyalgia.

OBJECTIVE: To assess the intraepidermal nerve fiber density in patients diagnosed with fibromyalgia (FM) and to evaluate the role of IgM binding to trisulfated heparin disaccharide (TS-HDS) in these patients. METHODS: FM is a poorly understood pain disorder with several proposed pathophysiologic mechanisms. It is characterized by widespread pain, fatigue, and sleep abnormalities. Small fiber neuropathy (SFN) has been proposed as an underlying mechanism, and patients with FM have been shown to have a reduction in the intraepidermal nerve fiber density. An underlying inflammatory process that could be a result of autoimmune phenomena has also been suggested. Non-length-dependent SFN (NLDSFN) has been shown to have a higher incidence of autoimmune disease. Twenty-two patients with established diagnosis of FM underwent skin biopsy at 2 sites; 10 cm above the lateral malleolus and 10 cm above the patella. Serum IgM binding to TS-HDS was assayed using an ELISA method. RESULTS: A total of 5/22 patients had positive TS-HDS antibodies; of these, 4 had NLDSFN (P = 0.0393). Comparison with a control group at Washington University showed no significant difference in percentage with TS-HDS antibodies (P = 0.41). When compared with Washington University database of skin biopsy, there was a trend for an increased percentage of NLDSFN in patients with FM (P = 0.06). CONCLUSIONS: This study further supports the hypothesis that a subgroup of patients with FM has SFN. We suggest a correlation between the presence of NLDSFN and TS-HDS antibodies.

Inflammatory Myopathies.

Idiopathic inflammatory myopathies are relatively rare diseases. Polymyositis and dermatomyositis are more common in women than men (2:1 ratio), while inclusion body myositis is twice as common in men. Inflammatory myopathies are a heterogeneous group of chronic systemic autoimmune diseases with an annual incidence of two to five cases per million, characterized by muscle inflammation and progressive muscle weakness. There are three major diseases which includes Dermatomyositis (DM) including a distinct juvenile subtype (JDM), Polymyositis (PM), and Inclusion Body Myositis. DM is a compliment mediated microangiopathy affecting skin and muscle. PM and IBM are T-cell mediated disorders, where CD8 positive cytotoxic T cells invade muscle fibers expressing MHC class I antigens, this leading to fiber necrosis. In IBM, vacuolar formation with amyloid deposits are also present. This article summarizes the clinical, histochemical and immunological features as well as the treatment options of the inflammatory myopathies.