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      Granulomatosis With Polyangiitis: The Complexity of Clinical Manifestations, Therapeutic Challenges, and Complications of a Severe Multisystemic Case

      case-report
      Author(s): 1 , , 1 , 2 , 1
      Editor(s): ,
      Publication date (Electronic, pub):
      Publication date (Electronic, collection):
      Journal: Cureus
      Publisher: Cureus
      Keywords: vasculitis, corticotherapy, cyclophosphamide, klebsiella pneumoniae, influenza, renal dysfunction, anti-pr3, anca, granulomatosis with polyangiitis

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          Abstract

          We report a case of a 34-year-old male with severe multisystemic involvement (including the testis, musculoskeletal system, skin, upper respiratory tract, ocular system, peripheral nerves, abdomen, and kidney) due to granulomatosis with polyangiitis (GPA) and a high proteinase 3 (PR3)-antineutrophil cytoplasmic antibodies (PR3ANCA) titer. A renal biopsy showed pauci-immune glomerulonephritis (GN). Systemic corticotherapy combined with cyclophosphamide was chosen for induction therapy. During the induction phase, clinical deterioration occurred in the form of severe alveolar hemorrhage, leading to admission to the intensive care unit (ICU). Influenza A (H1N1) was detected in the respiratory tract. Furthermore, blood sampling revealed an invasive Klebsiella pneumoniae infection that persisted despite multiple antibiotic regimens. A CT scan showed splenic vascular compromise, assumed to be the primary source of the infection, with sustained improvement after splenectomy. Maintenance therapy included a tapering dose of corticotherapy for 36 months and azathioprine 100mg daily for five years, which achieved full and sustained remission. The patient has been in full remission for nine years, with mild renal sequelae, including proteinuria and secondary hypertension.

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          EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis.

          M G Yates, R A Watts, I. M. Bajema … (2016)
          In this article, the 2009 European League Against Rheumatism (EULAR) recommendations for the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) have been updated. The 2009 recommendations were on the management of primary small and medium vessel vasculitis. The 2015 update has been developed by an international task force representing EULAR, the European Renal Association and the European Vasculitis Society (EUVAS). The recommendations are based upon evidence from systematic literature reviews, as well as expert opinion where appropriate. The evidence presented was discussed and summarised by the experts in the course of a consensus-finding and voting process. Levels of evidence and grades of recommendations were derived and levels of agreement (strengths of recommendations) determined. In addition to the voting by the task force members, the relevance of the recommendations was assessed by an online voting survey among members of EUVAS. Fifteen recommendations were developed, covering general aspects, such as attaining remission and the need for shared decision making between clinicians and patients. More specific items relate to starting immunosuppressive therapy in combination with glucocorticoids to induce remission, followed by a period of remission maintenance; for remission induction in life-threatening or organ-threatening AAV, cyclophosphamide and rituximab are considered to have similar efficacy; plasma exchange which is recommended, where licensed, in the setting of rapidly progressive renal failure or severe diffuse pulmonary haemorrhage. These recommendations are intended for use by healthcare professionals, doctors in specialist training, medical students, pharmaceutical industries and drug regulatory organisations.
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            Granulomatosis with polyangiitis (Wegener): clinical aspects and treatment.

            Cloé Comarmond, Patrice Cacoub (2014)
            Granulomatosis with polyangiitis (GPA) is a systemic necrotizing vasculitis, which affects small- and medium-sized blood vessels and is often associated with cytoplasmic ANCA. GPA occurs in patients between 45 and 60 years old of both genders, and is rarely observed in blacks. The prevalence of GPA increases along a south-north gradient in Europe (20 to 150/million). The main clinical characteristics involve the upper and/or lower respiratory tract and kidneys. Ear, nose and throat manifestations with recurrent sinusitis and crusting rhinorrhea are usually severe. Lung nodules are frequently seen, sometimes excavated. Renal involvement is characterized by rapidly progressive necrotizing glomerulonephritis with extracapillary crescents. Limited forms of GPA predominantly affect the upper respiratory tract, whereas generalized forms of GPA include renal manifestations and/or alveolar hemorrhage and/or vital organ involvement with an altered general condition. The combination of immunosuppressant drugs and corticosteroids has converted this typically fatal illness into one in which 80% of patients achieve remission. However, despite considerable therapeutic progress over the last decades, relapses remain frequent (50% at 5 years), and maintenance treatment is now the main therapeutic challenge.
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              Pathology of human influenza revisited.

              Thijs Kuiken, Jeffery K Taubenberger (2008)
              The pathology of human influenza has been studied most intensively during the three pandemics of the last century, the last of which occurred in 1968. It is important to revisit this subject because of the recent emergence of avian H5N1 influenza in humans as well as the threat of a new pandemic. Uncomplicated human influenza virus infection causes transient tracheo-bronchitis, corresponding with predominant virus attachment to tracheal and bronchial epithelial cells. The main complication is extension of viral infection to the alveoli, often with secondary bacterial infection, resulting in severe pneumonia. Complications in extra-respiratory tissues such as encephalopathy, myocarditis, and myopathy occur occasionally. Sensitive molecular and immunological techniques allow us to investigate whether these complications are a direct result of virus infection or an indirect result of severe pneumonia. Human disease from avian influenza virus infections is most severe for subtype H5N1, but also has been reported for H7 and H9 subtypes. In contrast to human influenza viruses, avian H5N1 virus attaches predominantly to alveolar and bronchiolar epithelium, corresponding with diffuse alveolar damage as the primary lesion. Viremia and extra-respiratory complications appear to be more common for infections with avian H5N1 virus than with human influenza viruses. Further understanding and comparison of the pathology of human and avian influenza virus infections only can be achieved by directed and careful pathological analysis of additional influenza cases.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Cureus
                Journal ID (iso-abbrev): Cureus
                Journal ID (issn): 2168-8184
                Title: Cureus
                Publisher: Cureus (Palo Alto (CA) )
                ISSN (Electronic): 2168-8184
                Publication date (Electronic, pub): 14 October 2023
                Publication date (Electronic, collection): October 2023
                Volume: 15
                Issue: 10
                Electronic Location Identifier: e47031
                Affiliations
                [1 ] Internal Medicine, Hospital Egas Moniz, Lisbon, PRT
                [2 ] Internal Medicine, Hospital Pedro Hispano, Porto, PRT
                Author notes
                Article
                DOI: 10.7759/cureus.47031
                PMC ID: 10642917
                SO-VID: 220c829b-56a4-46dc-a2db-1e2058546667
                Copyright © Copyright © 2023, Monteiro et al.
                License:

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                Date accepted : 13 October 2023
                Categories
                Subject: Internal Medicine
                Subject: Rheumatology
                Subject: Infectious Disease

                Keywords: vasculitis,corticotherapy,cyclophosphamide,klebsiella pneumoniae,influenza,renal dysfunction,anti-pr3,anca,granulomatosis with polyangiitis
                Data availability:
                Keywords: vasculitis, corticotherapy, cyclophosphamide, klebsiella pneumoniae, influenza, renal dysfunction, anti-pr3, anca, granulomatosis with polyangiitis

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