scholarly journals Could COVID-19 Trigger Presentation or Exacerbation of Vasculitides? (A 14-Year-Old Girl with Newly Diagnosed GPA After COVID-19 Infection)

2021 ◽  
Vol In Press (In Press) ◽  
Author(s):  
Seyed Reza Raeeskarami ◽  
Seyyed Hosein Mousavi ◽  
Mohammad Ashouri ◽  
Parvin Akbariasbagh ◽  
Raheleh Assari ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Pediatric Population ◽  
Multiple Organ ◽  
Newly Diagnosed ◽  
Gi Tract ◽  
Case Presentation ◽  
Anca Associated Vasculitis ◽  
Organ Systems ◽  
Necrotizing Glomerulonephritis ◽  
St Elevation

Introduction: Granulomatosis with polyangiitis (GPA) is a kind of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), which involves small-to-medium-sized vessels. Besides, GPA usually involves the upper and lower respiratory tract, and also causes necrotizing glomerulonephritis. The involvement of the heart and gastrointestinal (GI) tract in GPA is unusual, and these are atypical places of this vasculitis. Case Presentation: A 14-year-old girl with a newly diagnosed GPA after afflicting with COVID-19 infection presented with ST-elevation Myocardial Infarction (MI), GI perforation, and intracranial hemorrhage. Conclusions: Although GPA is rare in the pediatric population, it might occur in this age group and could involve multiple organ systems.

scholarly journals ANCA-Associated Vasculitis with Cardiac Valve Vegetations in Two Teenage Males: A Case Report and Literature Review

2022 ◽  
Author(s):  
Alexandra Theisen ◽  
Martha Rodriguez
Keyword(s):  
Cardiac Involvement ◽  
Granulomatosis With Polyangiitis ◽  
Pediatric Population ◽  
Adult Population ◽  
High Dose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Newly Diagnosed ◽  
Cardiac Morbidity ◽  
Anca Associated Vasculitis ◽  
Cardiac Manifestations

Abstract Background: Anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis is a term used to describe systemic vasculitides that affect small and medium-sized blood vessels. The three types of ANCA-associated vasculitis (AAV) are Granulomatosis with Polyangiitis (GPA), formerly Wegener’s granulomatosis , Microscopic Polyangiitis (MPA), and Eosinophilic Granulomatosis with Polyangiitis (EGPA), formerly Churg-Strauss, with clinical presentation most frequently involving the respiratory tract, the kidney, the skin and the joints. ANCA-associated vasculitis is rare in children, with estimated prevalence of 3-4 per million, and even more rare is the manifestation of cardiac abnormalities secondary to ANCA-associated vasculitis in the pediatric population. Case Presentation: We present the cases of two teenage males who presented with cardiac valvular lesions secondary to Granulomatosis with Polyangiitis in addition to sinus, pulmonary, renal, and cutaneous involvement. These findings of cardiac valvular abnormalities in GPA have rarely been described in the literature in pediatrics. Both patients were treated with rituximab, high-dose methylprednisolone, and plasma exchange (PLEX) and showed improvement in their disease manifestations. Conclusions: A review of the literature revealed only five pediatric cases of ANCA-associated vasculitis with cardiac manifestations, and interestingly, three of the five had valvular involvement. Subsequent valvular involvement makes obtaining the diagnosis of ANCA-Associated Vasculitis very difficult due to concern for underlying infectious endocarditis and can lead to misdiagnosis given the rarity of cardiac involvement in ANCA-associated vasculitis. Routine echocardiogram is not always completed in newly diagnosed GPA, yet cardiac involvement can lead to severe consequences as was seen with our first patient in the form of thromboembolic stroke. We discuss the importance of keeping AAV on the differential when cardiac lesions are present as well as the importance of regular cardiac screening in newly diagnosed patients with AAV, as it is a major factor of cardiac morbidity and mortality in the adult population and can contribute substantially to management decisions.

scholarly journals POS0830 FACTORS INFLUENCING PATIENT-REPORTED OUTCOMES IN ANCA-ASSOCIATED VASCULITIS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 668.2-669
Author(s):  
S. Monti ◽  
P. Delvino ◽  
C. Klersy ◽  
G. Coppa ◽  
A. Milanesi ◽  
...  
Keyword(s):  
Disease Activity ◽  
Global Assessment ◽  
Patient Reported Outcomes ◽  
Granulomatosis With Polyangiitis ◽  
Disease Duration ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Anca Associated Vasculitis ◽  
Organ Systems ◽  
Damage Accrual ◽  
Patient Reported

Background:Patient-reported outcomes (PROs) are currently poorly integrated in the clinical evaluation of disease activity in patients with ANCA-associated vasculitis (AAV).Objectives:To assess the distribution of the Patient Global Assessment (PtGA) in patients with AAV in stable remission, and to identify correlates of PtGA; to assess the discordance between PtGA score and PhGA.Methods:Patients with a diagnosis of AAV [eosinophilic granulomatosis with polyangiitis, granulomatosis with polyangiitis, microscopic polyangiitis] in stable, complete remission (defined by a BVAS=0) and with a Physician Global Assessment (PhGA)=0 were included. A questionnaire including several aspects of disease captured by PROs was collected. PtGA on a 0-100 mm visual analogue scale (VAS) was assessed, with higher scores representing higher/worse levels of disease activity. Similarly, VAS for pain, chronic damage according to the patient’s opinion, general health (GH), fatigue, and sleep quality were collected. The worst symptom in the patient’s opinion affecting the overall assessment of disease activity was recorded. The Cragg Hurdle model was used to assess the predictors of PtGA.Results:65 patients were included, female 57%, mean age 61±12 years. Mean disease duration at enrollment was 8±6 years. Mean vasculitis damage index (VDI) was 4.4 ±2.3, with 45% of patients having a VDI ≥ 5. Despite having been classified as being in remission, PtGA was elevated in 37% of patients. We explored several correlates of PtGA. Higher degree of damage accrual (VDI) did not influence the patient’s evaluation of current disease activity. Similarly, we did not identify a correlation between older age, educational level, number of organ-systems involved, number of comorbidities, the number of previous major or minor relapses, higher disease duration, nor the type of AAV diagnosis (figure 1, panel A). Only sex significantly correlated with PtGA scores: 19 (51%) of female patients reported an elevated PtGA compared to only 5 (18%) of male (p=0.009). PtGA resulted to be significantly correlated with other (mostly modifiable) PROs including VAS pain, perception of the level of chronic damage accrual, GH, and fatigue (figure 1, panel B). The agreement between patients’ and physicians’ assessments of disease activity was 63%. Patients reported pain, followed by chronic respiratory symptoms to be the worst-experienced ongoing manifestations affecting their evaluation of disease activity.Conclusion:A significant proportion of patients with AAV considered to be in remission by the physician still declares to have persistent aspects of uncontrolled disease. PtGA is significantly influenced by persistent pain and fatigue, which warrant better assessment in the future.Disclosure of Interests:None declared

scholarly journals Pyoderma Gangrenosum-like ulceration as a presenting feature of pediatric Granulomatosis with Polyangiitis

2020 ◽  
Author(s):  
Rotem Semo Oz ◽  
Oluwakemi Onajin ◽  
Liora Harel ◽  
Rotem Tal ◽  
Tomas Dallos ◽  
...  
Keyword(s):  
Pyoderma Gangrenosum ◽  
Granulomatosis With Polyangiitis ◽  
Skin Lesions ◽  
Response To Treatment ◽  
Newly Diagnosed ◽  
Rare Presentation ◽  
Case Presentation ◽  
Appropriate Treatment ◽  
The Mean ◽  
Systemic Symptoms

Abstract Background: Granulomatosis with polyangiitis (GPA) is an anti-neutrophilic cytoplasmic antibody-associated vasculitis affecting small to medium-sized vessels and involves most commonly the kidneys and the respiratory tract. Skin involvement can be seen in up to 50% of children with GPA and is the initial presenting symptom in 7.7%. Pyoderma gangrenosum (PG)-like ulcers are rarely described as a skin manifestation in GPA and very few cases have been reported previously in children. Case presentation: We describe 3 new pediatric cases of GPA with PG-like ulcerations. The mean age at first symptom was 15 years. Two patients had PG-like ulceration as their initial presentation; additional symptoms eventually led to the diagnosis of GPA 2-24 months later. In 1 case, proteinase 3 (PR3) was negative when first tested, but converted to positive when systemic symptoms emerged, in the other 2 cases PR3 was positive at presentation. All 3 patients had prominent facial lesions. None of the patients responded to treatment with antibiotics or medications commonly used to manage PG, including corticosteroids and cyclosporine. All patients had excellent responses to rituximab. An electronic database literature review was performed and 4 previously reported cases were identified. We assessed the clinical characteristics, serology, and response to treatment of 4 previously reported and newly diagnosed cases. Conclusion: PG-like ulceration is a rare presentation of pediatric GPA which may precede classic systemic GPA symptoms. The predominance of facial ulcer, granulomatous and neutrophilic inflammation on skin biopsy and lack of response to PG treatments are characteristic of GPA-associated PG-like ulcers. Our review suggests that treatment with rituximab may be needed to improve the skin lesions. Recognizing that PG-like ulcerations can occur in pediatric GPA may result in timely diagnosis, appropriate treatment and improved prognosis.

scholarly journals Klippel-Trenaunay Syndrome Causing Life-Threatening GI Bleeding: A Case Report and Review of the Literature

2013 ◽  
Vol 2013 ◽  
pp. 1-6 ◽  
Author(s):  
Salih Samo ◽  
Muhammed Sherid ◽  
Husein Husein ◽  
Samian Sulaiman ◽  
Margaret Yungbluth ◽  
...  
Keyword(s):  
Vascular Malformations ◽  
Multiple Organ ◽  
Gi Bleeding ◽  
Blood Products ◽  
Review Of The Literature ◽  
Gi Tract ◽  
Organ Systems ◽  
Life Threatening ◽  
Klippel Trenaunay Syndrome ◽  
Congenital Syndrome

Klippel-Trenaunay syndrome (KTS) is a rare congenital syndrome of vascular malformations and soft tissue and bone hypertrophy. Vascular malformations can affect multiple organ systems. Involvement of the gastrointestinal (GI) tract is uncommon in KTS, but it can be a source of life-threatening bleeding. We report a case of a 32-year-old male with a known diagnosis of KTS who presented with a life-threatening rectal bleeding and was treated with proctosigmoidectomy and massive blood products transfusion. He expired after a long hospitalization. We then review the literature on KTS and management of some of its complications.

scholarly journals Safety and Efficacy of Mepolizumab in Patients with Eosinophilic Granulomatosis with Polyangiitis

2019 ◽  
Vol 2019 ◽  
pp. 1-6 ◽  
Author(s):  
Ravi Ranjan Pradhan ◽  
Gaurav Nepal ◽  
Shobha Mandal
Keyword(s):  
Side Effects ◽  
Granulomatosis With Polyangiitis ◽  
Multiple Organ ◽  
High Dose ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Sustained Remission ◽  
Organ Systems ◽  
Frequent Relapses ◽  
Systemic Glucocorticoids ◽  
Eosinophilic Granulomatosis

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of vasculitis disorder which involves multiple organ systems and is characterized by asthma, pulmonary infiltrates, sinusitis, neuropathy, and peripheral eosinophilia. It also has an effect on the heart, skin, kidneys, and gastrointestinal tract. Interlukin-5 (IL-5) is involved in maturation and activation of eosinophil, the production of which is increased in the EGPA. Treatments of EGPA are limited to systemic corticosteroids and immunomodulators. These drugs are associated with significant side effects. Besides this, the response of patients to these drugs may be disappointing. Frequent relapses, the need for long-term medium-to-high-dose glucocorticoid therapy, and failure to achieve remission are not uncommon findings. There is a need for noble agents that could reduce frequent relapses and the dose of systemic glucocorticoids and maintain a sustained remission without significant side effects. Mepolizumab is IL-5 antagonist and may have value in treating patients with EGPA. Therefore, we did a systematic review to evaluate the efficacy and safety of mepolizumab in patients with EGPA.

scholarly journals Pediatric Granulomatosis With Polyangiitis Mimicking IgA Vasculitis: A Case Report

2020 ◽  
Vol 13 ◽  
pp. 117954412096737
Author(s):  
Vadood Javadi Parvaneh ◽  
Arezoo Shirzani ◽  
Khosro Rahmani ◽  
Reza Shiari
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
High Titer ◽  
Renal Involvement ◽  
Iga Vasculitis ◽  
Case Presentation ◽  
Tissue Biopsy ◽  
Anca Associated Vasculitis ◽  
Wide Range ◽  
Time Of Presentation

Background: Granulomatosis with polyangiitis (GPA) is a systemic vasculitis of the upper and lower respiratory tract along with glomerulonephritis and is very rare in childhood. Its renal manifestations similarity with IgA vasculitis can be misleading. Case presentation: Herein, we report a 12-years-old girl with the clinical picture of IgA vasculitis and renal involvement at the time of presentation, over time, elevated cytoplasmic Anti-neutrophil Cytoplasmic Antibody (C-ANCA) and tissue biopsy confirmed GPA. Conclusion: In the case of a patient with an unusual presentation of IgA vasculitis, to some degree of suspicion, the GPA should be considered. Also, in approach to non-thrombocytopenic palpable petechia and purpura a wide range of differential diagnosis such as infections, ANCA associated vasculitis, and secondary vasculitis should be considered. Therefore, 2 effective method of GPA diagnosis, the high titer of C-ANCA test and tissue biopsy, should be considered simultaneously.

scholarly journals Increased Levels of Soluble ST2 in Patients with Active Newly Diagnosed ANCA-Associated Vasculitis

2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Z. Hladinova ◽  
Z. Hruskova ◽  
B. Svobodova ◽  
K. Malickova ◽  
V. Lanska ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Interleukin 1 ◽  
Serum Levels ◽  
Th2 Cells ◽  
Newly Diagnosed ◽  
Control Groups ◽  
Soluble St2 ◽  
Anca Associated Vasculitis ◽  
Specific Ligand ◽  
Receptor Family

Objective. ST2, a member of the interleukin-1 receptor family, is selectively expressed on Th2 cells and mediates important Th2 functions. IL-33 is a specific ligand of ST2. The aim of the study was to determine whether serum levels of soluble ST2 (sST2) or IL-33 predict activity of the disease in patients with ANCA-associated vasculitides (AAV).Methods. 139 AAV patients and 62 controls were studied. IL-33 and sST2 in the blood were measured with a commercially available ELISA.Results. Newly diagnosed AAV patients had higher sST2 levels than controls (P<0.01). Levels of sST2 were significantly higher in active newly diagnosed AAV patients than in patients with remission (P<0.001). IL-33 levels were higher in AAV patients than in the control groups (P=0.002). However, serum IL-33 levels were not increased in patients with active AAV compared to patients in remission. IL-33 levels were higher in patients with granulomatosis with polyangiitis than in patients with microscopic polyangiitis (P=0.012).Conclusions. Serum sST2, but not serum IL-33, may be a marker of activity in AAV patients.

ANCA-associated vasculitis

2020 ◽  
pp. 4556-4569
Author(s):  
David Jayne
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Multiple Organ ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Histological Lesion ◽  
Considerable Heterogeneity ◽  
Organ Systems ◽  
Strauss Syndrome ◽  
Acute And Chronic Inflammation ◽  
Eosinophilic Granulomatosis ◽  
Churg Strauss

The ANCA-associated vasculitides (AAV) are a grouping of three syndromes of acute and chronic inflammation characterized by their clinical and histological phenotypes, which are associated with circulating antineutrophil cytoplasm autoantibodies (ANCA). They comprise: granulomatosis with polyangiitis (GPA, formerly Wegener’s granulomatosis); microscopic polyangiitis (MPA); and eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome). The defining histological lesion is a microscopic vasculitis affecting arterioles, capillaries, or venules associated with few or no deposits of immunoglobulin or complement. Granulomata, involving or close by blood vessels, are commonly present in GPA. These diseases involve multiple organ systems with considerable heterogeneity in extent and severity of organ involvement between patients, and overlapping clinical and histological features between syndromes.

scholarly journals Pyoderma gangrenosum-like ulceration as a presenting feature of pediatric granulomatosis with polyangiitis

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Rotem Semo Oz ◽  
Oluwakemi Onajin ◽  
Liora Harel ◽  
Rotem Tal ◽  
Tomas Dallos ◽  
...  
Keyword(s):  
Pyoderma Gangrenosum ◽  
Granulomatosis With Polyangiitis ◽  
Skin Lesions ◽  
Response To Treatment ◽  
Proteinase 3 ◽  
Newly Diagnosed ◽  
Rare Presentation ◽  
Case Presentation ◽  
Appropriate Treatment ◽  
Systemic Symptoms

Abstract Background Granulomatosis with polyangiitis (GPA) is an anti-neutrophilic cytoplasmic antibody-associated vasculitis affecting small to medium-sized vessels and involves most commonly the kidneys and the respiratory tract. Skin involvement can be seen in up to 50% of children with GPA and is the initial presenting symptom in 7.7%. Pyoderma gangrenosum (PG)-like ulcers are rarely described as a skin manifestation in GPA and very few cases have been reported previously in children. Case presentation We describe 3 new pediatric cases of GPA with PG-like ulcerations. The median age at first symptom was 15 years. Two patients had PG-like ulceration as their initial presentation; additional symptoms eventually led to the diagnosis of GPA 2–24 months later. In 1 case, proteinase 3 (PR3) was negative when first tested, but converted to positive when systemic symptoms emerged; in the other 2 cases PR3 was positive at presentation. All 3 patients had prominent facial lesions. None of the patients responded to treatment with antibiotics or medications commonly used to manage PG, including corticosteroids and cyclosporine. All patients had excellent responses to rituximab. An electronic database literature review was performed and 4 previously reported cases were identified. We assessed the clinical characteristics, serology, and response to treatment of the previously reported and our newly diagnosed cases. Conclusion PG-like ulceration is a rare presentation of pediatric GPA which may precede classic systemic GPA symptoms. The predominance of facial ulcer, granulomatous and neutrophilic inflammation on skin biopsy and lack of response to PG treatments are characteristic of GPA-associated PG-like ulcers. Our review suggests that treatment with rituximab may be needed to improve the skin lesions. Recognizing that PG-like ulcerations can occur in pediatric GPA may result in timely diagnosis, appropriate treatment and improved prognosis.

scholarly journals COVID-19 Multisystem Inflammatory Syndrome in Children (MIS-C) simulating as acute appendicitis: A case report

2020 ◽  
Vol 1 (1) ◽  
Author(s):  
Zorays Moazzam ◽  
Areej Salim ◽  
Kiran Hilal ◽  
Muhammad Arshad
Keyword(s):  
Abdominal Pain ◽  
Pediatric Patients ◽  
Acute Abdominal Pain ◽  
Multiple Organ ◽  
Fatal Complication ◽  
Case Presentation ◽  
Organ Systems ◽  
Surgical Conditions ◽  
Global Affairs ◽  
Inflammatory Syndrome

Background: As the COVID-19 pandemic continues to disrupt global affairs through a variety of manifestations, a severe presentation of some COVID-19 pediatric patients has emerged, presenting as multisystem inflammatory syndrome in children (MIS-C) that may mimic surgical conditions. Case Presentation: We report the case of a 9-year-old boy who presented with acute, intermittent abdominal pain and fever. Further inquiry suggested a hyperinflammatory disorder affecting multiple organ systems. Upon COVID-19 PCR testing, the patient was reported as COVID-19 positive in the second test on the 2nd day of admission, after an initial negative test upon admission. We describe the course of this patient’s disease from presentation until discharge and discuss the emerging literature on this potentially fatal complication. Conclusion: MIS-C should be a differential diagnosis in patients who may present with acute abdominal pain in the emergency room.

Sign in / Sign up

Export Citation Format

Share Document