scholarly journals Atypical Bacterial Pathogens and Small-Vessel Leukocytoclastic Vasculitis of the Skin in Children: Systematic Literature Review

Pathogens ◽  
2021 ◽  
Vol 10 (1) ◽  
pp. 31
Author(s):  
Céline Betti ◽  
Pietro Camozzi ◽  
Viola Gennaro ◽  
Mario G. Bianchetti ◽  
Martin Scoglio ◽  
...  
Keyword(s):  
Mycoplasma Pneumoniae ◽  
Legionella Pneumophila ◽  
Leukocytoclastic Vasculitis ◽  
Systemic Vasculitis ◽  
Bacterial Pathogen ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Systemic Involvement ◽  
Symptomatic Disease ◽  
Atypical Pathogen

Leukocytoclastic small-vessel vasculitis of the skin (with or without systemic involvement) is often preceded by infections such as common cold, tonsillopharyngitis, or otitis media. Our purpose was to document pediatric (≤18 years) cases preceded by a symptomatic disease caused by an atypical bacterial pathogen. We performed a literature search following the Preferred Reporting of Systematic Reviews and Meta-Analyses guidelines. We retained 19 reports including 22 cases (13 females and 9 males, 1.0 to 17, median 6.3 years of age) associated with a Mycoplasma pneumoniae infection. We did not find any case linked to Chlamydophila pneumoniae, Chlamydophila psittaci, Coxiella burnetii, Francisella tularensis, or Legionella pneumophila. Patients with a systemic vasculitis (N = 14) and with a skin-limited (N = 8) vasculitis did not significantly differ with respect to gender and age. The time to recovery was ≤12 weeks in all patients with this information. In conclusion, a cutaneous small-vessel vasculitis with or without systemic involvement may occur in childhood after an infection caused by the atypical bacterial pathogen Mycoplasma pneumoniae. The clinical picture and the course of cases preceded by recognized triggers and by this atypical pathogen are indistinguishable.

Systemic Vasculitis Syndromes

2017 ◽  
Author(s):  
Alexandra Villa-Forte ◽  
Brian F Mandell
Keyword(s):  
Kawasaki Disease ◽  
Blood Vessels ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Plain Radiograph ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Saddle Nose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Saddle Nose Deformity

Vasculitis is defined by histologic evidence of inflammation that involves the blood vessels. The diagnosis of a specific primary vasculitic disorder depends on the pattern of organ involvement, the histopathology, the size of affected blood vessels, and the exclusion of diseases that can cause “secondary” vasculitis. This review presents an approach to the patient suspected of having vasculitis, and goes on to discuss small vessel vasculitis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis, polyarteritis nodosa, Kawasaki disease, large vessel arteritis, and Behçet disease. Figures show classification of the systemic vasculitis syndromes, the relationships among the causes of small vessel (“hypersensitivity”) vasculitis, palpable purpura of the distal extremities, saddle nose deformity, the nodular infiltrates of the lung in granulomatosis with polyangiitis shown on plain radiograph as well as computed tomography, necrotizing scleritis, livedo reticularis, and angiograms of a patient with Takayasu arteritis. Tables list selected laboratory tests for patients with multisystem disease and possible vasculitis, practical comments on immunosuppressive therapies for vasculitis, features of vasculitis, diagnostic criteria for Kawasaki disease, and giant cell arteritis. This review contains 8 highly rendered figures, 5 tables, and 59 references.

scholarly journals P186 Efficacy and safety of rituximab in the treatment of systemic vasculitis: experience at a single centre

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_2) ◽  
Author(s):  
Carl Orr ◽  
Ayna Verdiyeva ◽  
Nadia Ahmad ◽  
Hairulhadi Ariff ◽  
Raashid Luqmani
Keyword(s):  
Systemic Vasculitis ◽  
Demographic Data ◽  
Underlying Disease ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Chimeric Antibody ◽  
Efficacy And Safety ◽  
Electronic Patient Records ◽  
Patient Demographic Data ◽  
Age Profile

Abstract Background Rituximab is a monoclonal chimeric antibody targeting the mature B cell molecule CD20, expressed on the cell surface. It is licenced and widely used in the treatment of autoimmune diseases, including the ANCA associated vasculitides and rheumatoid arthritis. The efficacy and safety of rituximab in rare diseases requires ongoing appraisal. Methods We interrogated the records of 62 consecutive patients treated with rituximab for small vessel systemic vasculitis at our unit over the last 3 years, using a contemporaneously maintained central database, as well as individual electronic patient records. Results The patient demographic data, and main results are presented in Table 1. 31 (50%) of the patients treated with rituximab were female, and the indication for treatment in most cases was ANCA associated vasculitis (77.4%). After a mean follow up period of 2.04 years, the mean reduction in BVAS was 1.64 (3.83). 5 severe adverse events were recorded, defined as death or hospitalisation potentially related to treatment with rituximab. 3 patients died of respiratory sepsis, 1 experienced a wound infection, and 1 experienced an ischaemic colon. Four patients were noted to have low immunoglobulins. Conclusion Rituximab is an effective treatment for many patients with systemic, small vessel vasculitis. Understanding the safety profile of this drug in the context of our cohort is challenging, given the significant morbidity associated with the underlying disease, as well as the age profile and concomitant immunosuppressive medications. Continued surveillance regarding efficacy and safety of rituximab in the small vessel systemic vasculitides remains invaluable. Disclosures C. Orr None. A. Verdiyeva None. N. Ahmad None. H. Ariff None. R. Luqmani None.

scholarly journals Mycoplasmal upper respiratory infection presenting as leukocytoclastic vasculitis

2015 ◽  
Vol 7 (1) ◽  
Author(s):  
Mana Rao ◽  
Abhinav Agrawal ◽  
Manan Parikh ◽  
Rikka Banayat ◽  
Maria Joana Thomas ◽  
...  
Keyword(s):  
Respiratory Tract ◽  
Leukocytoclastic Vasculitis ◽  
Small Vessel ◽  
Stevens Johnson Syndrome ◽  
Small Vessel Vasculitis ◽  
Upper Respiratory Tract ◽  
Cutaneous Lesions ◽  
Upper Respiratory Infection ◽  
Mycoplasmal Infection ◽  
Upper Respiratory

Mycoplasma is a virulent organism that is known to primarily infect the respiratory tract; however, affection of the skin, nervous system, kidneys, heart and bloodstream has been observed in various forms, which include Stevens Johnson syndrome, erythema multiforme, toxic epidermal necrolysis, encephalitis, renal failure, conduction system abnormalities and hemolytic anemia. Small vessel vasculitis is a lesser-known complication of mycoplasma pneumonia infection. We report a case of mycoplasmal upper respiratory tract infection with striking cutaneous lesions as the presenting symptom. Mycoplasmal infection was confirmed by serology testing, skin biopsy was suggestive of leukocytoclastic vasculitis. This case brings forth an uncommon manifestation of mycoplasmal infection with extra-pulmonary affection, namely small vessel vasculitis.

scholarly journals Case Report: Systemic Small-Vessel Vasculitis in an Adolescent With Active Ulcerative Colitis

2021 ◽  
Vol 9 ◽  
Author(s):  
Marleen Bouhuys ◽  
Wineke Armbrust ◽  
Patrick F. van Rheenen
Keyword(s):  
Ulcerative Colitis ◽  
Abdominal Pain ◽  
Gastrointestinal Tract ◽  
Leukocytoclastic Vasculitis ◽  
Foot Drop ◽  
Small Vessel ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Small Vessel Vasculitis ◽  
Induction Treatment ◽  
High Dose

Introduction: Small-vessel vasculitis (SVV) is a rare immunological disease that affects arterioles, capillaries and venules. It causes purpura, but can also manifest in other organs, including the gastrointestinal tract. SVV and inflammatory bowel disease (IBD) co-occur more frequently than would be expected by chance.Case description: A 16-year-old girl, who had been diagnosed with ulcerative colitis (UC) 2 years earlier at a general hospital, developed purpura, progressive abdominal pain with frequent bloody diarrhea and frontotemporal headache and swelling while on azathioprine and mesalamine maintenance therapy. Serology was positive for perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) without antiprotease- or myeloperoixidase antibodies. Endoscopy revealed active left-sided UC and atypical ulcerations in the ascending colon. Biopsies of these ulcerations and of affected skin revealed leukocytoclastic vasculitis. Initially this was interpreted as an extraintestinal manifestation of UC that would subside when remission was induced, consequently infliximab was started. Over the next 3 weeks she developed severe burning pain in her right lower leg that progressed to a foot drop with numbness and the purpura progressed to bullous lesions. The diagnosis was adjusted to ANCA-associated vasculitis with involvement of skin, bowel and peripheral nerves. Infliximab was discontinued and induction treatment with high-dose prednisolone and cyclophosphamide was given until remission of SVV and UC was achieved. Subsequently, infliximab induction and maintenance was re-introduced in combination with methotrexate. Remission has been maintained successfully for over 2 years now. The foot drop only partly resolved and necessitated the use of an orthosis.Conclusion: Pediatric patients with IBD who present with purpuric skin lesions and abdominal pain should be evaluated for systemic involvement of SVV, which includes endoscopic evaluation of the gastrointestinal tract. We discuss a practical approach to the diagnosis, evaluation and management of systemic SVV with a focus on prompt recognition and early aggressive therapy to improve outcome.

scholarly journals The Diagnostic and Clinical Utility of Autoantibodies in Systemic Vasculitis

Antibodies ◽  
2019 ◽  
Vol 8 (2) ◽  
pp. 31 ◽  
Author(s):  
Elena Csernok
Keyword(s):  
Systemic Vasculitis ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Urticarial Vasculitis ◽  
International Consensus ◽  
Anca Associated Vasculitis ◽  
Distinct Disease ◽  
A Current ◽  
Made In

Considerable progress has been made in understanding the role of autoantibodies in systemic vasculitides (SV), and consequently testing for anti-neutrophil cytoplasmic antibodies (ANCA), anti-glomerular basement membrane antibodies (anti-GBM), and anti-C1q antibodies is helpful and necessary in the diagnosis, prognosis, and monitoring of small-vessel vasculitis. ANCA-directed proteinase 3 (PR3-) or myeloperoxidase (MPO-) are sensitive and specific serologic markers for ANCA-associated vasculitides (AAV), anti-GBM antibodies are highly specific for the patients with anti-GBM antibody disease (formerly Goodpasture’s syndrome), and autoantibodies to C1q are characteristic of hypocomlementemic urticarial vasculitis syndrome (HUVS; anti-C1q vasculitis). The results of a current EUVAS study have led to changes in the established strategy for the ANCA testing in small-vessel vasculitis. The revised 2017 international consensus recommendations for ANCA detection support the primary use PR3- and MPO-ANCA immunoassays without the categorical need for additional indirect immunofluorescence (IIF). Interestingly, the presence of PR3- and MPO-ANCA have led to the differentiation of distinct disease phenotype of AAV: PR3-ANCA-associated vasculitis (PR3-AAV), MPO-ANCA-associated vasculitis (MPO-AAV), and ANCA-negative vasculitis. Further studies on the role of these autoantibodies are required to better categorize and manage appropriately the patients with small-vessel vasculitis and to develop more targeted therapy.

scholarly journals Leukocytoclastic vasculitis (cutaneous small-vessel vasculitis) after COVID-19 vaccination

2021 ◽  
pp. 102783
Author(s):  
G. Fiorillo ◽  
S. Pancetti ◽  
A. Cortese ◽  
F. Toso ◽  
S. Manara ◽  
...  
Keyword(s):  
Leukocytoclastic Vasculitis ◽  
Small Vessel ◽  
Small Vessel Vasculitis

scholarly journals AB0098 ASSOCIATED FACTORS IN ONE-YEAR MORTALITY IN PATIENTS WITH SMALL VESSEL SYSTEMIC VASCULITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1078.2-1078
Author(s):  
E. M. Escudero Tepale ◽  
M. A. Saavedra ◽  
D. Miranda ◽  
Z. Castro ◽  
M. J. R. Arriga Torres
Keyword(s):  
Associated Factors ◽  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Remission Induction ◽  
First Year ◽  
Factors Associated ◽  
Number Of Patients ◽  
One Year

Background:The highest mortality rates in patients with small vessel systemic vasculitis occur within the first year after the diagnosis, however associated factors have been scarcely studied in our population.Objectives:To identify mortality associated factors at the time of diagnosis in patients with small vessel systemic vasculitis.Methods:Retrospective cohort (2009-2020) involving 81 patients diagnosed with systemic small vessel vasculitis. Demographic, clinical and biochemical parameters were studied as potential factors associated with one-year mortality.Results:Of the total of patients (n=81), 36 (44.4%) had generalized granulomatosis with polyangiitis, 32 (39.5%) had localized granulomatosis with polyangiitis and 5 (6.2%) had early systemic granulomatosis with polyangiitis, 7 (8.6%) had microscopic polyangiitis and 1 (1.25%) had eosinophilic granulomatosis with polyangiitis. Twenty-two deaths (27%) were observed, 14 of them (63.6%) happened within the first year of diagnosis. The leading cause of death was infection (64%). Patients who died within the first year of diagnosis had a higher frequency of hypoalbuminemia (p=0.05) and also presented hemoglobin lower than 10.8 g/dL (p=0.035) in comparison with those who died after the first year of diagnosis. Remission induction treatment did not differ between both groups.Conclusion:Our study suggests that hypoalbuminemia and anemia are factors associated with a higher mortality within the first year after the diagnosis in patients with systemic small vessel vasculitis which contrast with previously reported data. The study design and the reduced number of patients are two major limitations of the study.References:[1]Flossmann O, Berden A, Groot K, et al. Long-term patient survival in ANCA-associated vasculitis. Ann Rheum Dis. 2011;70:488-94.Disclosure of Interests:None declared

scholarly journals Henoch-Schonlein Purpura in Children: The Role of Corticosteroids

2019 ◽  
Vol 7 (11) ◽  
pp. 1812-1814
Author(s):  
Bella Kurnia
Keyword(s):  
Abdominal Pain ◽  
Renal Disease ◽  
Systemic Vasculitis ◽  
Small Vessel ◽  
Small Vessel Vasculitis ◽  
Henoch Schonlein Purpura ◽  
Progression Of Renal Disease ◽  
Schonlein Purpura ◽  
Henoch Schönlein Purpura

BACKGROUND: Henoch- schonlein purpura (HSP) is an IgA- mediated systemic small vessel vasculitis. It is the most common form of systemic vasculitis in children.CASE REPORT: A 9 years old girl admitted to the hospital with chief complain of purplish red rash on both legs since approximately 1 week with painful knees and ankles that make the patient unable to walk. The patient was diagnosed with HSP and was treated with corticosteroid and analgesics. The patients only stayed for 2 nights at the hospital and discharged from the hospital with the ability to walk and experience no pain. CONCLUSION: The role of corticosteroids in the treatment of HSP is still controversial. But from various research, we can conclude that the role of corticosteroid in HSP is as a symptom reliever (reduce abdominal pain and arthritis), but does not slow the progression of renal disease.

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