Polymyositis, Pulmonary Fibrosis and Malignant Lymphoma Associated with Hepatitis C Virus Infection.

Thoracic manifestations of the rheumatic diseases (RDs) are a significant cause of morbidity and mortality worldwide. The five RDs most frequently associated with pleuropulmonary disease are (1) rheumatoid arthritis (RA), (2) systemic lupus erythematosus (SLE), (3) progressive systemic sclerosis (PSS), (4) polymyositis/dermatomyositis (PM/DM), and (5) Sjögren syndrome (SS). The clinical presentation is highly variable, ranging from pleuritic pain alone to slowly progressive breathlessness accompanied by cough. On occasion RD may present acutely with overwhelming respiratory failure as the first pulmonary manifestation. In all of the RDs, the pathology is dominated by either or both inflammation and fibrosis, but the anatomical distribution in each varies somewhat. For example, airway-associated lymphoid hyperplasia is a common manifestation of SS, PM/DM preferentially involves the alveolar parenchyma, and pleural inflammation is most commonly seen in RA. Such changes may be detected radiologically as peribronchovascular disease in SS, ground-glass infiltrates in PM/DM, and pleural disease with effusion in RA. Some RDs are more commonly associated with distinctive histopathology, such as rheumatoid nodules in RA, or lymphoid hyperplasia in both RA and SS. Patients with PSS are at particular risk for pulmonary hypertension when the lung is involved. Also, statistically, acute lung injury occurs more commonly in some, such as SLE and PM/DM. The very common use of immunosuppressive agents in the treatment of RD creates additional diagnostic problems related to drug toxicity and infection. Finally, a major confounding factor occurs when preclinical RD presents first as pulmonary disease (particularly PM/DM), an occurrence that may invoke one of the so-called idiopathic interstitial pneumonias. The most common pleuropulmonary pathological manifestations of the five major RDs are presented and discussed with brief radiological correlations.

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“…59,60 Patients with PM/DM may be at increased risk of developing lung lymphoma and carcinoma. 19,61 SJÖ GREN SYNDROME…”

Section: Figure 13mentioning

confidence: 99%

Pulmonary Pathology of the Rheumatic Diseases

Leslie¹,

Trahan

Gruden

2007

Semin Respir Crit Care Med

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“…In all GBV-B infected animals, CK was elevated up to 18-fold above baseline. Multiple studies have founded elevated CK and myositis associated with HCV infection [89–92]. This is further supported by the gross pathological observations of hepatomegaly, splenomegaly, kidney lesions and peripheral lymphadenopathy–indicative of an underlying state of inflammation caused by GBV-B infection and similarity to HCV disease.…”

Section: Discussionmentioning

confidence: 78%

Metabolic Dysregulation in Hepacivirus Infection of Common Marmosets (Callithrix jacchus)

et al. 2017

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Chronic hepatitis C has been associated with metabolic syndrome that includes insulin resistance, hepatic steatosis and obesity. These metabolic aberrations are risk factors for disease severity and treatment outcome in infected patients. Experimental infection of marmosets with GBV-B serves as a tangible, small animal model for human HCV infection, and while virology and pathology are well described, a full investigation of clinical disease and the metabolic milieu is lacking. In this study six marmosets were infected intravenously with GBV-B and changes in hematologic, serum biochemical and plasma metabolic measures were investigated over the duration of infection. Infected animals exhibited signs of lymphocytopenia, but platelet and RBC counts were generally stable or even increased. Although most animals showed a transient decline in blood glucose, infection resulted in several fold increases in plasma insulin, glucagon and glucagon-like peptide 1 (GLP-1). All infected animals experienced transient weight loss within the first 28 days of infection, but also became hypertriglyceridemic and had up to 10-fold increases in adipocytokines such as resistin and plasminogen activator inhibitor 1 (PAI-1). In liver, moderate to severe cytoplasmic changes associated with steatotic changes was observed microscopically at 168 days post infection. Collectively, these results suggest that GBV-B infection is accompanied by hematologic, biochemical and metabolic abnormalities that could lead to obesity, diabetes, thrombosis and atherosclerosis, even after virus has been cleared. Our findings mirror those found in HCV patients, suggesting that metabolic syndrome could be conserved among hepaciviruses, and both mechanistic and interventional studies for treating HCV-induced metabolic complications could be evaluated in this animal model.

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“…Polymyositis is frequently reported in HCV infection, either without [77, 78] or with CG [79], being associated with interstitial lung disease in some patients. The presence of genomic HCV RNA, but not replicative intermediates in muscle specimens, a picture suggestive of an autoimmune HCV-triggered process, has been detected in patients with polymyositis [80, 81].…”

Section: Myopathiesmentioning

confidence: 99%

HCV-Related Nervous System Disorders

Monaco

Ferrari

Gajofatto

et al. 2012

Clinical and Developmental Immunology

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Chronic infection with hepatitis C virus (HCV) is associated with a wide spectrum of extrahepatic manifestations, affecting different organ systems. Neurological complications occur in a large number of patients and range from peripheral neuropathy to cognitive impairment. Pathogenetic mechanisms responsible for nervous system dysfunction are mainly related to the upregulation of the host immune response with production of autoantibodies, immune complexes, and cryoglobulins. Alternative mechanisms include possible extrahepatic replication of HCV in neural tissues and the effects of circulating inflammatory cytokines and chemokines.

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Polymyositis, Pulmonary Fibrosis and Malignant Lymphoma Associated with Hepatitis C Virus Infection.

Cited by 20 publications

References 24 publications

Pulmonary Pathology of the Rheumatic Diseases

Pulmonary Pathology of the Rheumatic Diseases

Metabolic Dysregulation in Hepacivirus Infection of Common Marmosets (Callithrix jacchus)

HCV-Related Nervous System Disorders

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