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      Azathioprine therapy for steroid-resistant Henoch-Schönlein purpura: a report of 6 cases

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          Abstract

          Background

          A small percentage of children with Henoch-Schönlein purpura (HSP) develop a chronic form of the disease that often requires prolonged corticosteroid therapy. Disease modifying anti-rheumatic agents (DMARDs) or biologics have been successfully used to treat those refractory cases. Azathioprine is a DMARD that has been reported to be effective in HSP nephritis and in adult cutaneous leukocytoclastic vasculitis, a condition with cutaneous histology similar to HSP.

          Case presentation

          A description of 6 cases with relapsing HSP without significant renal involvement, treated with azathioprine are reported. All 6 cases met the classification criteria for the diagnosis of HSP, had relapsing symptoms despite corticosteroid use, were successfully treated with azathioprine and were tapered off of corticosteroids. The duration of azathioprine therapy ranged from 7–21 months and no adverse events were reported.

          Conclusions

          Azathioprine is effective in controlling prolonged relapsing symptoms of HSP, allowing earlier discontinuation of corticosteroids. This report shows that azathioprine can be included in the therapeutic options for relapsing HSP and is the first case series in the literature of azathioprine use in HSP without significant renal involvement.

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          Most cited references27

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          Early prednisone therapy in Henoch-Schönlein purpura: a randomized, double-blind, placebo-controlled trial.

          To evaluate the efficacy of early prednisone therapy in preventing renal and treating extrarenal and renal symptoms in Henoch-Schönlein purpura (HSP) in a placebo-controlled trial. A total of 171 patients (84 treated with prednisone and 87 receiving placebo) were included and followed up for 6 months. The endpoints were renal involvement at 1, 3, and 6 months and healing of extrarenal symptoms. The analyses were performed on an intent-to-treat basis. Prednisone (1 mg/kg/day for 2 weeks, with weaning over the subsequent 2 weeks) was effective in reducing the intensity of abdominal pain (pain score, 2.5 vs 4.8; P = .029) and joint pain (4.6 vs 7.3; P = .030). Prednisone did not prevent the development of renal symptoms but was effective in treating them; renal symptoms resolved in 61% of the prednisone patients after treatment, compared with 34% of the placebo patients (difference = 27%; 95% confidence interval = 3% to 47%; P = .024). The general use of prednisone in HSP is not supported, but patients with disturbing symptoms may benefit from early treatment, because prednisone reduces extrarenal symptoms and is effective in altering (but not preventing) the course of renal involvement.
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            Vasculitis in systemic lupus erythematosus: prevalence and clinical characteristics in 670 patients.

            We conducted the current study to determine the prevalence and clinical characteristics of vasculitis in a large series of patients with systemic lupus erythematosus (SLE), focusing on the classification and clinical significance of the different types of vasculitis. We studied 670 consecutive patients who fulfilled 4 or more of the 1997 revised criteria for SLE. Definite vasculitis was diagnosed histologically and/or by arteriography, and probable vasculitis was diagnosed clinically when there were characteristic cutaneous lesions. Vasculitides were categorized according to the definitions adopted by the Chapel Hill Consensus Conference. Seventy-six (11%) patients with SLE had vasculitis (68 female patients and 8 male; mean age, 37.8 yr); only 32 (42%) fulfilled the Chapel Hill definitions. Cutaneous lesions were the main clinical presentation of vasculitis, present in 68 (89%) patients, while the remaining 8 (11%) had isolated visceral vasculitis. Compared with SLE patients without vasculitis, patients with vasculitis had a higher prevalence of livedo reticularis (22% vs. 3%; p = 0.028); a higher mean European Consensus Lupus Activity Measurement (ECLAM) score (5.86 vs. 3.87; p 50 mm/h (60% vs. 15%; p < 0.001), and anti-La/SS-B antibodies (19% vs. 5%; p = 0.014) in the multivariate analysis. With respect to the size of the vessels involved, 65 (86%) patients had small vessel vasculitis (SVV) and 11 (14%) had medium-sized vessel vasculitis (MVV). SLE patients with MVV had a higher prevalence of mononeuritis multiplex (54% vs. 2%; p < 0.001), visceral vasculitis (100% vs. 5%; p < 0.001), and ulcerated/ischemic cutaneous lesions (36% vs. 11%; p = 0.047) and a higher percentage of surgical interventions (45% vs. 0%; p < 0.001) compared with patients with SVV. In conclusion, we observed a heterogeneous presentation of vasculitides arising in the setting of SLE, with nearly 60% of cases not fulfilling the names and definitions adopted by the Chapel Hill Consensus Conference. SVV was the most frequent vasculitis, overwhelmingly cutaneous and clearly differentiated from MVV, which was less frequent but had predominantly visceral involvement (especially of the peripheral nerves). The presence of vasculitis in our patients with SLE was associated with a higher ECLAM score, livedo reticularis, hematologic parameters (anemia, high ESR), and anti-La/SS-B antibodies.
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              Effective therapy for severe Henoch-Schonlein purpura nephritis with prednisone and azathioprine: a clinical and histopathologic study.

              To validate a scoring system to assess the severity of renal lesions and to correlate histology with clinical findings. We also examined the efficacy of treatment with prednisone (1 to 2 mg/kg/d) and azathioprine (1 to 2 mg/kg/d) for severe Henoch-Schonlein purpura (HSP) nephritis. Twenty patients were evaluated retrospectively. All underwent biopsy before treatment, and 13 underwent biopsy after therapy. We developed a scale based on glomerular, tubulointerstitial (TI), and vascular changes and assigned all specimens acuity, chronicity, and TI scores. The outcomes of 17 patients were compared with those of a historical control group. Chronicity score at initial biopsy increased with increasing delay between onset of renal involvement and first biopsy (rho = 0.55, P =.016) but did not progress after treatment was initiated. Both acuity (rho = 0.57,P =. 016) and TI (rho = 0.69, P =.003) scores correlated with clinical severity at first biopsy. The TI score correlated negatively with serum albumin (rho = -.60, P <.01). Significantly more patients in the study group than in the control group had a favorable outcome (15 [88%] of 17 vs 32 [54%] of 59, P =.011). Our scale reflects disease activity and highlights the importance of TI changes in severe HSP nephritis. Outcome comparisons indicate that early treatment with prednisone and azathioprine prevents progression of chronic changes and improves outcome.
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                Author and article information

                Contributors
                +1-314-454-6124 , +1-314-454-4861 , fotis_l@kids.wustl.edu
                ptuttlei@slu.edu
                baszis_k@kids.wustl.edu
                pepmueph@slu.edu
                mooretl@slu.edu
                white@kids.wustl.edu
                Journal
                Pediatr Rheumatol Online J
                Pediatr Rheumatol Online J
                Pediatric Rheumatology Online Journal
                BioMed Central (London )
                1546-0096
                23 June 2016
                23 June 2016
                2016
                : 14
                : 37
                Affiliations
                [ ]Department of Pediatrics, Division of Rheumatology, Washington University School of Medicine, One Children’s Place, Campus Box 8116, St. Louis, MO 63110 USA
                [ ]Department of Internal Medicine, Division of Rheumatology and Pediatric Rheumatology, Saint Louis University School of Medicine, 1402 S Grand Blvd., Doisy Hall, Room R213A, St. Louis, MO 63104 USA
                Article
                100
                10.1186/s12969-016-0100-x
                4918135
                27333803
                de0b003e-8270-4bb3-8153-dbc270ba7f52
                © The Author(s). 2016

                Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

                History
                : 23 March 2016
                : 15 June 2016
                Funding
                Funded by: No financial interest to disclose
                Categories
                Case Report
                Custom metadata
                © The Author(s) 2016

                Pediatrics
                purpura, schoenlein-henoch,azathioprine,steroids,child
                Pediatrics
                purpura, schoenlein-henoch, azathioprine, steroids, child

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