Recurrent gastrointestinal bleeding due to jejunal artery vasculitis as debut presentation of granulomatosis with polyangiitis

Granulomatosis with polyangiitis (GPA) is characterised by systemic necrotising vasculitis of small arteries and veins with multitude of organ involvement, with the most common being the upper and lower respiratory tract and renal system. Gastrointestinal involvement is a rare late manifestation with a high mortality rate and usually results in intestinal perforation. Our patient presented with gastrointestinal bleeding secondary to jejunal artery vasculitis. Gastrointestinal bleeding as initial presentation of GPA is very rarely documented. CT mesenteric angiogram is helpful for the localisation of bleed in these cases. In case of refractory bleeding, surgical excision is required.

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Temporal trend and survival impact of infection source among patients with sepsis: a nationwide study

Critical Care and Resuscitation ◽

10.51893/2020.2.oa2 ◽

2020 ◽

Vol 22 (2) ◽

pp. 126-132

Author(s):

Yueh-Sheng Chen ◽

◽

Tin-Yun Liao ◽

Tzu-Chun Hsu ◽

Wan-Ting Hsu ◽

...

Keyword(s):

Mortality Rate ◽

Tract Infection ◽

Respiratory Tract ◽

Lower Respiratory Tract ◽

Temporal Trends ◽

Administrative Database ◽

Common Source ◽

Sepsis Management ◽

Incidence And Mortality ◽

Different Sources

BACKGROUND: To determine the temporal trends of incidence and outcome based on different sources of sepsis using a nationwide administrative database. METHODS: From 2002 to 2012, the entire Taiwan's health insurance claims data of emergency-treated and hospital-treated sepsis were analysed for incidence and mortality trends. The information about patients with sepsis and sources of sepsis was identified using a set of validated International Classification of Diseases, ninth revision, clinical modification (ICD-9-CM) codes. The 30-day all-cause mortality was verified by linked death certificate database. RESULTS: A total of 1 259 578 episodes of sepsis were identified during the 11-year study period. Lower respiratory tract infection is the most common source of sepsis in patients, with the highest mortality rate. The incidence of genitourinary tract infection has the fastest growing rate. The sepsis mortality was declining at different rates for each source of sepsis. Co-infections in patients with sepsis are associated with higher mortality rate. CONCLUSION: The temporal trends of sepsis incidence and mortality varied among different sources of sepsis, with lower respiratory tract being the highest burden among patients with sepsis. Furthermore, sources of sepsis and the presence of co-infection are independent predictors of mortality. Our results support source-specific preventive and treatment strategies for future sepsis management.

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Clinical features of ANCA-associated vasculitis

Oxford Textbook of Rheumatology ◽

10.1093/med/9780199642489.003.0131_update_001 ◽

2013 ◽

pp. 1090-1102

Author(s):

Wolfgang L. Gross ◽

Julia U. Holle

Keyword(s):

Respiratory Tract ◽

Clinical Features ◽

Lower Respiratory Tract ◽

Granulomatosis With Polyangiitis ◽

Systemic Vasculitis ◽

Systemic Disease ◽

Clinical Signs ◽

Eosinophilic Granulomatosis With Polyangiitis ◽

Granulomatous Lesions ◽

Organ Manifestations

The primary ANCA-associated vasculitides are granulomatosis with polyangiitis (Wegener’s, GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome, CSS). They predominantly affect small (and medium-sized) vessels and share a variable association with ANCA (anti-neutrophil cytoplasm antibody) directed against neutrophil proteinase 3 (PR3, mainly in GPA) and myeloperoxidase (MPO, mainly in MPA and CSS). Crescentic necrotizing glomerulonephritis and alveolar haemorrhage due to pulmonary capillaritis represent classical (vasculitic) organ manifestations of the ANCA-associated vasculitides (AAV). MPA occurs as a ’pure’ small (to medium-size) vessel vasculitis, whereas GPA and CSS are characterized by additional distinct clinical and pathological features. In GPA, granulomatous lesions of the upper and/or lower respiratory tract are a hallmark of the disease. Granulomatous lesions may be large in appearance and occur as space-consuming, infiltrating, and destructive inflammatory masses. GPA is believed to follow a stagewise course with an initial localized form, restricted granulomatous lesions of the upper and/or lower respiratory tract without clinical signs of vasculitis, and a consecutive generalization to systemic vasculitis which may be either non-organ-threatening (early systemic) or organ- and life- threatening (generalized GPA). Rarely, patients arrest in the localized stage and do not progress to systemic disease. In EGPA asthma, hypereosinophilia and eosinophilic organ infiltration (e.g. eosinophilic myocarditis) are typical features of the disease apart from vasculitis. Similarly to GPA, EGPA follows a stagewise course: asthma and eosinophilia may precede full-blown disease for several months or years. Recent cohort studies suggest different phenotypes in EGPA (predominantly vasculitic and MPO-ANCA-positive and predominantly with eosinophilic organ infiltration, usually ANCA-negative). This chapter focuses on the clinical features of the primary AAV and their outcome.

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Clinical features of ANCA-associated vasculitis

10.1093/med/9780199642489.003.0131 ◽

2013 ◽

Author(s):

Wolfgang L. Gross ◽

Julia U. Holle

Keyword(s):

Respiratory Tract ◽

Clinical Features ◽

Lower Respiratory Tract ◽

Granulomatosis With Polyangiitis ◽

Systemic Disease ◽

Alveolar Haemorrhage ◽

Medium Size ◽

Eosinophilic Granulomatosis With Polyangiitis ◽

Necrotizing Glomerulonephritis ◽

Granulomatous Lesions

The primary ANCA-associated vasculitides are granulomatosis with polyangiitis (Wegener's, GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome, CSS). They predominantly affect small (and medium-sized) vessels and share a variable association with ANCA (anti-neutrophil cytoplasm antibody) directed against neutrophil proteinase 3 (PR3, mainly in GPA) and myeloperoxidase (MPO, mainly in MPA and CSS). Crescentic necrotizing glomerulonephritis and alveolar haemorrhage due to pulmonary capillaritis represent classical (vasculitic) organ manifestations of the ANCA-associated vasculitides (AAV). MPA occurs as a 'pure' small (to medium-size) vessel vasculitis, whereas GPA and CSS are characterized by additional distinct clinical and pathological features. In GPA, granulomatous lesions of the upper and/or lower respiratory tract are a hallmark of the disease. Granulomatous lesions may be large in appearance and occur as space-consuming, infiltrating, and destructive inflammatory masses. GPA is believed to follow a stagewise course with an initial localized form, restricted granulomatous lesions of the upper and/or lower respiratory tract without clinical signs of vasculitis, and a consecutive generalization to systemic vasculitis which may be either non-organ-threatening (early systemic) or organ- and life- threatening (generalized GPA). Rarely, patients arrest in the localized stage and do not progress to systemic disease. In EGPA asthma, hypereosinophilia and eosinophilic organ infiltration (e.g. eosinophilic myocarditis) are typical features of the disease apart from vasculitis. Similarly to GPA, EGPA follows a stagewise course: asthma and eosinophilia may precede full-blown disease for several months or years. Recent cohort studies suggest different phenotypes in EGPA (predominantly vasculitic and MPO-ANCA-positive and predominantly with eosinophilic organ infiltration, usually ANCA-negative). This chapter focuses on the clinical features of the primary AAV and their outcome.

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062. UPPER AND LOWER RESPIRATORY TRACT INVOLVEMENT IS SIMILAR IN MPO-ANCA AND PR3-ANCA GRANULOMATOSIS WITH POLYANGIITIS

Rheumatology ◽

10.1093/rheumatology/kez058.002 ◽

2019 ◽

Vol 58 (Supplement_2) ◽

Author(s):

Marco Antonio Alba Garibay ◽

Susan Hogan ◽

Ashley Henderson ◽

Caroline Poulton ◽

Yichun Hu ◽

...

Keyword(s):

Respiratory Tract ◽

Lower Respiratory Tract ◽

Granulomatosis With Polyangiitis ◽

Tract Involvement

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Variation in Mortality Rates Among Long-Term Care Facilities for Residents With Lower Respiratory Tract Infection

Infection Control and Hospital Epidemiology ◽

10.1086/590123 ◽

2008 ◽

Vol 29 (8) ◽

pp. 754-759 ◽

Cited By ~ 4

Author(s):

L. E. Nicolle ◽

S. Mubareka ◽

A. Simor ◽

B. Liu ◽

S. McNeil ◽

...

Keyword(s):

Mortality Rate ◽

Tract Infection ◽

Respiratory Tract ◽

Respiratory Tract Infection ◽

Lower Respiratory Tract Infection ◽

Lower Respiratory Tract ◽

Long Term Care ◽

Term Care ◽

Care Facilities

Objective.To identify variables contributing to interfacility differences in mortality among residents of long-term care facilities who have lower respiratory tract infection.Design.Multicenter, prospective, 1 -year observational study.Setting.Twenty-one long-term care facilities in 4 geographic areas of Canada.Participants.Residents of long-term care facilities prescribed antimicrobials for treatment of lower respiratory tract infection.Methods.Mortality rates were calculated for 3 definitions of lower respiratory tract infection: episodes with a clinical or radiographic diagnosis and treated with antimicrobials (definition 1); episodes with a physician diagnosis of pneumonia (definition 2); and episodes with chest radiography findings consistent with pneumonia (definition 3). Multilevel modeling was used to evaluate variables describing premorbid resident status, clinical presentation, management, and facility characteristics. Multivariable models were developed to identify independent predictors of mortality and determine whether facility-level variables remained independently associated with mortality rate after incorporation of individual-level variables.Results.Facility mortality rates varied from 0% to 17.8% for definition 1, from 0% to 47.1% for definition 2, and from 0% to 37.5% for definition 3. There were significant differences in mortality rate depending on which definition was used; for definitions 1 and 2, there were significant differences in mortality rate across facilities. Poorer premorbid resident status and a more severe presentation remained independent predictors of mortality in the multivariable analysis. There were also significantly increased mortality rates for episodes in which a fluoroquinolone was prescribed for initial treatment. For definitions 1 and 3, facility-level variables remained independently associated with mortality rate in the final multivariable model.Conclusions.Rates of mortality due to lower respiratory tract infection varied among long-term care facilities and differed within a facility, depending on the definition applied. Variables describing premorbid resident status, severity of presentation, and management did not fully explain the variation in mortality rate. Some facility-level variables remained independent predictors of mortality.

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Gastrointestinal Hemorrhage in Patient with Granulomatosis with Polyangitis

Case Reports in Critical Care ◽

10.1155/2021/9921361 ◽

2021 ◽

Vol 2021 ◽

pp. 1-3

Author(s):

Nikhil Madan ◽

Vipul Patel

Keyword(s):

Gastrointestinal Tract ◽

Gastrointestinal Hemorrhage ◽

Lower Respiratory Tract ◽

Rare Presentation ◽

Bowel Infarction ◽

Small Arteries ◽

Gastrointestinal Manifestations ◽

Granulomatous Vasculitis ◽

Pulmonary Renal Syndrome ◽

Arteries And Veins

Granulomatosis with polyangitis (GPA) is characterized by a necrotizing granulomatous vasculitis of small arteries and veins. It most commonly affects the upper and lower respiratory tract and kidneys. However, other organs including the gastrointestinal tract can be affected. Gastrointestinal manifestations of GPA are rare and can include ischemia, bowel infarction, and perforation. Hemorrhage is an extremely rare presentation of GPA. We present a case of a woman with GPA and pulmonary renal syndrome on treatment who presents with severe gastrointestinal hemorrhage.

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An Unusual Presentation of Limited Granulomatosis with Polyangiitis Involving Vagina and Urethra

Case Reports in Rheumatology ◽

10.1155/2017/9407675 ◽

2017 ◽

Vol 2017 ◽

pp. 1-3 ◽

Cited By ~ 1

Author(s):

Sandra Soro Marín ◽

Enrique Júdez Navarro ◽

Manuela Sianes Fernández ◽

Ginés Sánchez Nievas ◽

Juan Gabriel Lorenzo Romero

Keyword(s):

Respiratory Tract ◽

Blood Vessels ◽

Lower Respiratory Tract ◽

Granulomatosis With Polyangiitis ◽

Unusual Presentation ◽

Multisystemic Disease ◽

Irregular Mass

Granulomatosis with polyangiitis (GPA) is a systemic necrotizing granulomatosis vasculitis characterized by predilection to affect small- and medium-sized blood vessels and commonly affects the upper and lower respiratory tract and kidneys in most cases. Genital involvement is reported in <1% of cases in large cohorts and nearly all cases have been in the setting of multisystemic disease or during the course of the disease in patients already diagnosed as GPA. A case is presented of uncommon limited urogenital GPA in a 66-year-old woman with an irregular mass occupying urethra and vagina. The patient showed a good response after Corticoids and Methotrexate.

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