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      Paraneoplastic IgA Vasculitis in an Adult with Lung Adenocarcinoma

      case-report

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          Abstract

          A 50-year-old man with lung adenocarcinoma (c-T1aN2M1b) experienced reddish purpura mainly on the lower legs after receiving 12 cycles of second-line chemotherapy with docetaxel. There was tumor enlargement on computed tomography performed to assess the therapeutic response, so paraneoplastic IgA vasculitis was considered. IgA vasculitis was diagnosed based on a biopsy of the skin lesion and histology of an upper gastrointestinal hemorrhagic mucosal erosion. As IgA vasculitis can lead to serious gastrointestinal or systemic complications, IgA vasculitis should be considered as a differential diagnosis for rashes in patients with malignancy.

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          The American College of Rheumatology 1990 criteria for the classification of Henoch-Schönlein purpura.

          Christina Michel, Blair Leavitt, A Fauci … (1990)
          Criteria for identifying Henoch-Schönlein Purpura (HSP) and distinguishing HSP from other forms of systemic arteritis were developed by comparing the manifestations in 85 patients who had HSP with those of 722 control patients with other forms of vasculitis. By the traditional format of choosing different combinations of candidate criteria and comparing the combinations for their ability to separate HSP cases from controls, 4 criteria were identified: age less than or equal to 20 years at disease onset, palpable purpura, acute abdominal pain, and biopsy showing granulocytes in the walls of small arterioles or venules. The presence of any 2 or more of these criteria distinguish HSP from other forms of vasculitis with a sensitivity of 87.1% and a specificity of 87.7%. The criteria selected by a classification tree method were similar: palpable purpura, age less than or equal to 20 years at disease onset, biopsy showing granulocytes around arterioles or venules, and gastrointestinal bleeding. These were able to distinguish HSP from other forms of vasculitis with a sensitivity of 89.4% and a specificity of 88.1%.
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            Paraneoplastic vasculitis in patients with solid tumors: report of 15 cases.

            Roser Solans, C Aznar, Cathy A J Bosch … (2008)
            To review all cases of concurrent vasculitis and solid tumors diagnosed at our Department over a 15-year period and explore evidence that would support the notion of vasculitis being a true paraneoplastic syndrome. We reviewed the records of all patients diagnosed with vasculitis and solid tumors within 12 months of each other and prospectively followed until death or our report. We analyzed the main features and outcome of vasculitis in this setting. We also reviewed all cases published in the French-English literature. Fifteen patients (9 men and 6 women) in whom both vasculitis and solid tumor occurred within the same 12 months were identified. Mean age was 72.5 years (range 58-84). In 7 cases the diagnosis of vasculitis antedated that of cancer, in 6 both processes were synchronously diagnosed, and in 2 vasculitis appeared after cancer diagnosis. The most common vasculitis was cutaneous leukocytoclastic vasculitis (n = 9). Other vasculitides included Henoch-Shönlein purpura (n = 2), polyarteritis nodosa (n = 1), and giant cell arteritis (n = 3). The commonest malignancies were carcinomas of urinary organs (40%), lung (26.7%), and gastrointestinal tract (26.7%). The median followup was 28.4 months (range 1-96). Thirteen of the 15 patients demonstrated concordance of disease activity and treatment response for both cancer and vasculitis. Vasculitis flared heralding tumor recurrence or progression in 7 (46.6%) cases. In our patients, resolution of vasculitis following effective treatment of the putatively linked malignancy, and recurrence of vasculitis heralding tumor recurrence or progression, provide strong evidence for vasculitis being a true paraneoplastic syndrome. Chronic or persistent vasculitis with poor response to usually effective therapy, especially in elderly patients, should raise questions about underlying malignancy.
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              A clinical study of Henoch-Schönlein Purpura associated with malignancy.

              S Shimada, H. Mitsui, T Kawamura … (2009)
              Malignancy has been reported as a causative factor of cutaneous vasculitis, although only two retrospective epidemiological studies have analysed the association between Henoch-Schönlein purpura (HSP) and malignancy to date. To analyse the association between adult HSP and malignancy. We retrospectively reviewed the medical records of patients and found 103 cases of HSP over the past 20 years. Fifty-three cases (aged > or = 41 years) were categorized to two groups including 'with malignancy' or 'without malignancy', so that we could analyse the differences of clinical features between them. We also compared our study to previous reports. Twenty-three cases out of 53 patients exhibited underlying malignant tumours. We focused on nine patients in which malignant tumours were thought to be strongly associated. Seven of nine patients exhibited new metastatic lesions or died due to underlying cancer within 1-32 months. An association between HSP and malignant disease might have important diagnostic and pathophysiologic implications.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Intern Med
                Journal ID (iso-abbrev): Intern. Med
                Title: Internal Medicine
                Publisher: The Japanese Society of Internal Medicine
                ISSN (Print): 0918-2918
                ISSN (Electronic): 1349-7235
                Publication date (Electronic): 27 December 2017
                Publication date (Print): 1 May 2018
                Volume: 57
                Issue: 9
                Pages: 1273-1276
                Affiliations
                [1 ]Division of Medical Oncology, Teikyo University School of Medicine, Japan
                [2 ]Division of Pathology, Teikyo University School of Medicine, Japan
                Author notes

                Correspondence to Dr.　Nobuhiko Seki, nseki@ 123456med.teikyo-u.ac.jp

                Article
                DOI: 10.2169/internalmedicine.9651-17
                PMC ID: 5980809
                PubMed ID: 29279496
                SO-VID: 034ccc30-2975-45e0-a144-b512680f5a4a
                Copyright © Copyright © 2018 by The Japanese Society of Internal Medicine
                License:

                The Internal Medicine is an Open Access article distributed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. To view the details of this license, please visit ( https://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                Date received : 13 June 2017
                Date accepted : 31 August 2017
                Categories
                Subject: Case Report

                Keywords: iga vasculitis,lung cancer,paraneoplastic vasculitis,drug eruption
                Data availability:
                Keywords: iga vasculitis, lung cancer, paraneoplastic vasculitis, drug eruption

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