Management of Interstitial Lung Disease in Patients With Myositis Specific Autoantibodies. 2018

Christopher A Mecoli, and Lisa Christopher-Stine
Division of Rheumatology, Johns Hopkins University School of Medicine, 5200 Eastern Ave, Mason F. Lord, Center Tower; Suite 4100, Baltimore, MD, 21224, USA.

To review advances in the management of idiopathic inflammatory myopathy-associated interstitial lung disease (IIM-associated ILD) in the past 5 years, with highlights in myositis-specific antibody (MSA) groups. With the recent advent of widespread MSA testing, the study of specific homogeneous autoantibody-based subgroups of IIM-associated ILD is now possible. The prevalence, severity, prognosis, and response to treatment are under study for these individual MSAs. Early evidence suggests that PL-7- and PL-12-positive patients are more likely to have ILD and worse severity, compared to Jo-1 patients. Many medications have been efficacious for the treatment of IIM-associated ILD, including calcineurin inhibitors, rituximab, and cyclophosphamide. We suggest vigilant screening and monitoring of ILD in IIM patients with focus on the potential side effects associated with therapy and thus advocate appropriate vaccination, PCP prophylaxis, and bone health protection. Many different agents are used to manage patients with ILD with no comparative effectiveness studies to guide the clinician. The possibility of using MSAs to help guide treatment decisions is an appealing, although unproven, focus of research. Unfortunately, the rarity of non-Jo-1 myositis-specific antibodies has precluded robust study of response to treatment and overall management. Ongoing clinical trials and working groups are coordinating efforts to provide evidence-based management.

UI MeSH Term Description Entries
D007166 Immunosuppressive Agents Agents that suppress immune function by one of several mechanisms of action. Classical cytotoxic immunosuppressants act by inhibiting DNA synthesis. Others may act through activation of T-CELLS or by inhibiting the activation of HELPER CELLS. While immunosuppression has been brought about in the past primarily to prevent rejection of transplanted organs, new applications involving mediation of the effects of INTERLEUKINS and other CYTOKINES are emerging. Immunosuppressant,Immunosuppressive Agent,Immunosuppressants,Agent, Immunosuppressive,Agents, Immunosuppressive
D009173 Mycophenolic Acid Compound derived from Penicillium stoloniferum and related species. It blocks de novo biosynthesis of purine nucleotides by inhibition of the enzyme inosine monophosphate dehydrogenase (IMP DEHYDROGENASE). Mycophenolic acid exerts selective effects on the immune system in which it prevents the proliferation of T-CELLS, LYMPHOCYTES, and the formation of antibodies from B-CELLS. It may also inhibit recruitment of LEUKOCYTES to sites of INFLAMMATION. Cellcept,Mycophenolate Mofetil,Mycophenolate Mofetil Hydrochloride,Mycophenolate Sodium,Mycophenolic Acid Morpholinoethyl Ester,Myfortic,RS 61443,RS-61443,Sodium Mycophenolate,Mofetil Hydrochloride, Mycophenolate,Mofetil, Mycophenolate,Mycophenolate, Sodium,RS61443
D009220 Myositis Inflammation of a muscle or muscle tissue. Inflammatory Myopathy,Myositis, Focal,Myositis, Infectious,Idiopathic Inflammatory Myopathies,Idiopathic Inflammatory Myopathy,Idiopathic Inflammatory Myositis,Infectious Myositis,Inflammatory Muscle Diseases,Inflammatory Myopathies, Idiopathic,Inflammatory Myopathy, Idiopathic,Muscle Diseases, Inflammatory,Myopathies, Idiopathic Inflammatory,Myopathy, Inflammatory,Myositis, Proliferative,Focal Myositides,Focal Myositis,Infectious Myositides,Inflammatory Muscle Disease,Inflammatory Myopathies,Muscle Disease, Inflammatory,Myopathies, Inflammatory,Myopathy, Idiopathic Inflammatory,Myositides,Myositides, Focal,Myositides, Infectious,Myositides, Proliferative,Proliferative Myositides,Proliferative Myositis
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000069283 Rituximab A murine-derived monoclonal antibody and ANTINEOPLASTIC AGENT that binds specifically to the CD20 ANTIGEN and is used in the treatment of LEUKEMIA; LYMPHOMA and RHEUMATOID ARTHRITIS. CD20 Antibody, Rituximab,GP2013,IDEC-C2B8,IDEC-C2B8 Antibody,Mabthera,Rituxan,IDEC C2B8,IDEC C2B8 Antibody,Rituximab CD20 Antibody
D001323 Autoantibodies Antibodies that react with self-antigens (AUTOANTIGENS) of the organism that produced them. Autoantibody
D001327 Autoimmune Diseases Disorders that are characterized by the production of antibodies that react with host tissues or immune effector cells that are autoreactive to endogenous peptides. Autoimmune Disease,Disease, Autoimmune,Diseases, Autoimmune
D001379 Azathioprine An immunosuppressive agent used in combination with cyclophosphamide and hydroxychloroquine in the treatment of rheumatoid arthritis. According to the Fourth Annual Report on Carcinogens (NTP 85-002, 1985), this substance has been listed as a known carcinogen. (Merck Index, 11th ed) Azathioprine Sodium,Azathioprine Sodium Salt,Azathioprine Sulfate,Azothioprine,Immuran,Imuran,Imurel,Sodium, Azathioprine
D017563 Lung Diseases, Interstitial A diverse group of lung diseases that affect the lung parenchyma. They are characterized by an initial inflammation of PULMONARY ALVEOLI that extends to the interstitium and beyond leading to diffuse PULMONARY FIBROSIS. Interstitial lung diseases are classified by their etiology (known or unknown causes), and radiological-pathological features. Diffuse Parenchymal Lung Disease,Diffuse Parenchymal Lung Diseases,Interstitial Lung Disease,Interstitial Lung Diseases,Pneumonia, Interstitial,Pneumonitis, Interstitial,Interstitial Pneumonia,Interstitial Pneumonias,Interstitial Pneumonitides,Interstitial Pneumonitis,Lung Disease, Interstitial,Pneumonias, Interstitial,Pneumonitides, Interstitial
D065095 Calcineurin Inhibitors Compounds that inhibit or block the PHOSPHATASE activity of CALCINEURIN. Calcineurin Inhibitor,Calcineurin Antagonists,Calcineurin Blockers,Protein Phosphatase 3 Inhibitors,Protein Phosphatase-2B Inhibitors,Inhibitor, Calcineurin,Protein Phosphatase 2B Inhibitors

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