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      Etiological and clinicopathological study of secondary small vessel vasculitis in elderly: A case series of 12 patients

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          Abstract

          Background:

          Inflammation involving the postcapillary venular wall is defined as small vessel vasculitis. Small vessel vasculitis has various clinical manifestations. Etiologically, it can be primary or secondary. Literature regarding secondary vasculitis in elderly is scanty.

          Aim and Objectives:

          In this case series, we aimed to assess the clinical features and etiologies of biopsy-proven secondary small vessel vasculitis in the elderly.

          Methodology:

          Twelve elderly patients with biopsy-proven small vessel vasculitis were included in this study. All patients were thoroughly evaluated to assess the etiology and presence of major organ involvement.

          Results:

          Secondary small vessel vasculitis involved both the sexes equally. Constitutional symptoms including fever and weight loss were noticed by most of the (70%) patients. Neurological deficits were present in 83% of the study population. The most common finding in an electromyographic examination was an asymmetric sensory motor distal predominant polyradiculopathy. Fifty percent of the patients did fulfill the criteria for a definite autoimmune disease. More than 30% of the vasculitis was secondary to malignancies.

          Conclusions:

          Neurological manifestations are the most common systemic involvement in elderly patients with secondary vasculitis. Meticulous search for underlying malignancies is mandatory in elderly patients with secondary small vessel vasculitis.

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          Most cited references19

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          Epidemiology of systemic vasculitis: a ten-year study in the United Kingdom.

          To describe the epidemiology of the primary systemic vasculitides (PSV; Wegener's granulomatosis, Churg-Strauss syndrome, microscopic polyangiitis, polyarteritis nodosa) in a well-defined population over a 10-year period. An inception cohort of patients from the Norwich Health Authority (NHA) who were >15 years of age and had PSV first diagnosed between January 1, 1988 and December 31, 1997 was collected. Incidence rates were adjusted for age and sex to the 1992 population. The prevalence of PSV in this cohort was estimated on December 31, 1997. Patients were classified according to the American College of Rheumatology 1990 vasculitis criteria and the Chapel Hill Consensus definitions. Eighty-two NHA residents fulfilled the inclusion criteria. There were 47 men and 35 women, with a mean age of 62.9 years (median 65.0 years). The overall annual incidence of PSV among NHA residents was 19.8/million (95% confidence interval [95% CI] 15.8-24.6). The point prevalence on December 31, 1997 was 144.5/million (95% CI 110.4-185.3). PSV was more common in males (23.5/million; 95% CI 17.3-31.3) than females (16.4/million; 95% CI 11.4-22.8). The age- and sex-specific incidence showed a clear increase with age, with an overall peak in the 65-74 year age group (60.1/million). In our study population, the annual incidence of PSV is slowly increasing with time and the incidence is greatest in the elderly.
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            Diagnostic approach to patients with suspected vasculitis.

            E Suresh (2006)
            Vasculitis presents several diagnostic challenges. Firstly, patients could present with protean clinical manifestations with a wide spectrum ranging from isolated cutaneous vasculitis to multisystem involvement. Secondly, there are several medical conditions that could mimic the presentation of vasculitis. The range of differential diagnosis is therefore broad. Thirdly, vasculitis could occur as a primary disorder or be secondary to various medical conditions. It becomes important to differentiate them, as treatment of some forms of vasculitis such as those that are secondary to infection or drugs, is different from that of primary vasculitis. Fourthly, there are several different forms of vasculitis. Some are benign and self limiting, while others have the potential to threaten vital organ function and life. It follows that a rational approach is required during evaluation of patients with suspected vasculitis.
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              Temporal concurrence of vasculitis and cancer: a report of 12 cases.

              Vasculitis has been associated with solid organ and hematologic cancer. The rarity of these associations, and in many reports the lack of temporal relationships, has led to skepticism about vasculitis being a paraneoplastic syndrome. The objective of the present study was to review cases of concurrent vasculitis and cancer at the Cleveland Clinic Foundation over an 18.5-year period and explore evidence that would support the notion of vasculitis being a type of paraneoplastic disease. Retrospective review of the records of all patients diagnosed with vasculitis and cancer within 12 months of each other was performed using an ICD-9 diagnostic data base at the Cleveland Clinic Foundation. Patients with known chronic autoimmune disease or serologic evidence of hepatitis B or C infection were excluded. A standardized data collection instrument was used to document information about presentation, treatment, and course of illness. During the 18.5 years of our study, more than 15 million inpatients and outpatients were seen at the Cleveland Clinic. Of these, 2,800 patients had vasculitis independent of cancer, more than 69,000 patients had cancer, and 69 patients had been identified who had both malignancies and systemic vasculitis. Only 12 patients were identified in whom both vasculitis and cancer occurred within the same 12 months. Mean age was 65 years (range 45-79). There was no gender preference (M = F). In 8 of the 12 cases, diagnoses were made within 3 months of each other. In 6 of the patients, the diagnoses of both processes were made within 1 month. Ten of the 12 patients had vasculitis 1 to 3 months prior to or concurrent with the diagnosis of cancer. Six of the 12 patients had solid organ tumors, 4 had lymphoma, 1 had leukemia, and 1 had multiple myeloma. The most common vasculitis was cutaneous leukocytoclastic vasculitis (LCV), which occurred in 7 cases. Four cases of LCV were associated with solid organ tumors. Other vasculitides included giant cell arteritis (n = 2), polyarteritis nodosa (n = 2), and Wegener's granulomatosis (n = 1). The response of the vasculitis to glucocorticoid and cytotoxic therapy varied. Complete remission of vasculitis occurred in 4 of the patients, partial improvement occurred in 4 patients, and no improvement was noted in 4 patients. Complete remission occurred in 3 of the 4 patients in whom vasculitis and cancer were treated concurrently. Eight of 10 patients in whom followup was greater than 2 months demonstrated concordance of disease activity and treatment response for both cancer and vasculitis. The close temporal relationship of cancer and vasculitis in our patients adds to circumstantial evidence of vasculitis at times being a paraneoplastic condition. Failure of a vasculitis to respond to conventional therapy should raise questions about underlying malignancy. Effective treatment of the cancer enhances the likelihood of improvement in vasculitis.
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                Author and article information

                Journal
                J Family Med Prim Care
                J Family Med Prim Care
                JFMPC
                Journal of Family Medicine and Primary Care
                Medknow Publications & Media Pvt Ltd (India )
                2249-4863
                2278-7135
                Jan-Mar 2017
                : 6
                : 1
                : 106-109
                Affiliations
                [1 ] Department of Internal Medicine, Christian Medical College and Hospital, Vellore, Tamil Nadu, India
                [2 ] Department of Dermatology, Christian Medical College and Hospital, Vellore, Tamil Nadu, India
                [3 ] Department of Geriatrics, Christian Medical College and Hospital, Vellore, Tamil Nadu, India
                Author notes
                Address for correspondence: Dr. Ajay Kumar Mishra, Department of Internal Medicine, Unit V, Hospital Campus, Christian Medical College, Vellore - 632 004, Tamil Nadu, India. E-mail: ajaybalasore@ 123456gmail.com
                Article
                JFMPC-6-106
                10.4103/2249-4863.214977
                5629871
                4d89cbb9-6fbe-4e5e-b70f-5d4e7827f298
                Copyright: © 2017 Journal of Family Medicine and Primary Care

                This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

                History
                Categories
                Original Article

                elderly,malignancy,neuropathy,secondary,vasculitis
                elderly, malignancy, neuropathy, secondary, vasculitis

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