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ACR Open Rheumatology Feb 2021The object of this study was to analyze the benefits and harms of different treatment options and to analyze test accuracy used in the evaluation of patients with...
OBJECTIVE
The object of this study was to analyze the benefits and harms of different treatment options and to analyze test accuracy used in the evaluation of patients with primary systemic polyarteritis nodosa (PAN).
METHODS
A systematic search of published English-language literature was performed in Ovid Medline, PubMed, Embase, and the Cochrane Library from the inception of each database through August 2019. Articles were screened for suitability in addressing patient, intervention, comparison, and outcome questions, with studies presenting the highest level of evidence given preference.
RESULTS
Of 137 articles selected for data abstraction, we analyzed 21 observational studies and seven randomized controlled trials (RCTs). The results showed indirect evidence that a deep skin biopsy provides good diagnostic accuracy. A combined nerve and muscle biopsy should be obtained for patients with PAN with peripheral neuropathy. Cyclophosphamide with high-dose glucocorticoids (GCs) is effective as an induction treatment for newly diagnosed active and severe PAN. GC monotherapy is adequate in the majority of patients with nonsevere PAN, although it has a high relapse rate with GC taper. There was insufficient data in determining the optimal duration of non-GC and GC maintenance therapy. Tumor necrosis factor inhibitors are effective treatment for patients with deficiency of adenosine deaminase 2 (DADA2) with stroke and vasculitis manifestations.
CONCLUSION
This comprehensive systematic review synthesizes and evaluates the harms and benefits of different treatment options and the accuracy of commonly used tests for the diagnosis of systemic PAN. Data for diagnosis and management of PAN and DADA2 are mostly limited to observational studies. More high-quality RCTs are needed.
PubMed: 33512781
DOI: 10.1002/acr2.11189 -
Annals of the Rheumatic Diseases Oct 2022
Tocilizumab for the treatment of polyarteritis nodosa: a systematic literature review. Correspondence on 'Tofacitinib for polyarteritis nodosa: a tailored therapy' by Rimar .
Topics: Antibodies, Monoclonal, Humanized; Humans; Piperidines; Polyarteritis Nodosa; Pyrimidines
PubMed: 32907804
DOI: 10.1136/annrheumdis-2020-218710 -
Medicina (Kaunas, Lithuania) Aug 2020Background and Objectives Over the last years, inflammatory bowel disease (IBD) has been reported on a high incidence in pediatric populations and has been associated...
UNLABELLED
Background and Objectives Over the last years, inflammatory bowel disease (IBD) has been reported on a high incidence in pediatric populations and has been associated with numerous extraintestinal manifestations, making its management a real challenge for the pediatric gastroenterologist. Dermatological manifestations in IBD are either specific, related to the disease activity or treatment-associated, or non-specific. This literature review aims to identify and report the dermatological manifestations of IBD in children, the correlation between their appearance and the demographical characteristics, the relationship between these lesions and disease activity, and to highlight the impact of dermatological manifestations on an IBD treatment regime.
MATERIALS AND METHODS
A systemic literature review was performed, investigating articles and case reports on dermatological manifestations in children with IBD starting from 2005. A total of 159 potentially suitable articles were identified and after the exclusion process, 75 articles were selected.
RESULTS
The most common dermatological manifestations reported in pediatric IBD are erythema nodosum and pyoderma gangrenosum. More rare cases of metastatic Crohn's disease, epidermolysis bullosa acquisita, small-vessel vasculitis, necrotizing vasculitis, leukocytoclastic vasculitis, cutaneous polyarteritis nodosa, and Sweet's syndrome have been reported. Oral manifestations of IBD are divided into specific (tag-like lesions, mucogingivitis, lip swelling with vertical fissures, aphthous stomatitis, and pyostomatitis vegetans) and non-specific. IBD treatment may present with side effects involving the skin and mucosa. Anti-tumor necrosis factor agents have been linked to opportunistic skin infections, psoriasiform lesions, and a potentially increased risk for skin cancer. Cutaneous manifestations such as acrodermatitis enteropathica, purpuric lesions, and angular cheilitis may appear secondary to malnutrition and/or malabsorption.
CONCLUSIONS
The correct diagnosis of dermatological manifestations in pediatric IBD is of paramount importance because of their impact on disease activity, treatment options, and a patient's psychological status.
Topics: Biological Factors; Child; Colitis, Ulcerative; Crohn Disease; Humans; Immunosuppressive Agents; Malabsorption Syndromes; Receptors, Tumor Necrosis Factor; Skin Diseases
PubMed: 32842528
DOI: 10.3390/medicina56090425 -
Frontiers in Immunology 2019Certain types of vasculitis occur more frequently and present differently in patients with familial Mediterranean fever (FMF). We assessed the characteristics of... (Meta-Analysis)
Meta-Analysis
Certain types of vasculitis occur more frequently and present differently in patients with familial Mediterranean fever (FMF). We assessed the characteristics of patients with FMF and systemic vasculitis through a systematic review of the literature. Medline was searched by two independent investigators until December 2017. We screened 310 articles and selected 58 of them (IgA vasculitis = 12, polyarteritis nodosa (PAN) = 25, Behçet's disease (BD) = 7, other vasculitis = 14). Clinical case reports were available for 167 patients (IgA vasculitis = 46, PAN = 61, BD = 46, other vasculitis = 14), and unavailable for 45 patients (IgA vasculitis = 38, PAN = 7). IgA vasculitis was the most common vasculitis in FMF patients with a prevalence of 2.7-7%, followed by PAN with a prevalence of 0.9-1.4%. Characteristics of FMF did not differ between patients with and without vasculitis. Patients with FMF and IgA vasculitis displayed more intussusception (8.7%) and possibly less IgA deposits on histological analysis than patients with IgA vasculitis alone. Patients with FMF and PAN had a younger age at vasculitis onset (mean age = 17.9 years), as well as more perirenal hematomas (49%) and CNS involvement (31%) than patients with PAN alone. Glomerular involvement was noted in 33% of patients diagnosed with PAN, suggesting an alternative diagnosis. Sequencing of the gene confirmed the presence of two pathogenic variants in 73% of FMF patients with IgA vasculitis or PAN. The majority of patients with BD were from one case series, and presented more skin, gastrointestinal, and CNS involvement than patients with isolated BD. In conclusion, FMF, particularly when supported by two pathogenic mutations, could predispose to IgA vasculitis, or a PAN-like vasculitis with more perirenal bleeding and CNS involvement.
Topics: Age of Onset; Disease Management; Disease Susceptibility; Familial Mediterranean Fever; Humans; Phenotype; Prevalence; Sex Factors; Vasculitis
PubMed: 31031761
DOI: 10.3389/fimmu.2019.00763 -
International Urology and Nephrology Nov 2017To conduct a systematic literature review on spontaneous renal hemorrhage (SRH) in a contemporary cohort describing patterns in etiology and treatment. (Review)
Review
OBJECTIVE
To conduct a systematic literature review on spontaneous renal hemorrhage (SRH) in a contemporary cohort describing patterns in etiology and treatment.
METHODS
A systematic search of MEDLINE and CENTRAL databases was conducted to include articles, including case reports and case series on SRH published from 2000 to 2016. Full-text manuscripts were reviewed for clinical parameters which were collated and analyzed with univariate methods.
RESULTS
Seventy-nine publications met inclusion criteria, reporting on 102 cases. Renal neoplasms (56.9%) and polyarteritis nodosa (PAN) (11.8%) remained as the most common overall and vascular causes of SRH, respectively. Angiomyolipoma (AML) was the most common causative renal neoplasm (74.1%), and patients were more likely to be female and present with macroscopic hematuria than those with vasculitis, while malignant neoplasms were more common in men. Proportions of SRH due to malignant neoplasms (specifically renal cell carcinoma, RCC) were reported less than PAN. Among this contemporary series, transarterial embolization (TAE) was most commonly used for acute SRH (42.2%).
CONCLUSIONS
Renal neoplasms remain as the most common cause of SRH, of which AML predominates, while PAN is currently the second most common etiology in acute SRH, replacing RCC. Minimally invasive approaches, such as TAE and conservative/medical management, were preferred to initial surgery.
SYSTEMATIC REVIEW REGISTRATION
PROSPERO registration number CRD42017069222.
Topics: Angiomyolipoma; Carcinoma, Renal Cell; Embolization, Therapeutic; Hematuria; Hemorrhage; Humans; Kidney Neoplasms; Polyarteritis Nodosa; Sex Factors
PubMed: 28871505
DOI: 10.1007/s11255-017-1694-8 -
Clinical Rheumatology Nov 2016To investigate the possible association between systemic vasculitis and risk of venous thromboembolism (VTE), two investigators independently searched published studies... (Meta-Analysis)
Meta-Analysis Review
To investigate the possible association between systemic vasculitis and risk of venous thromboembolism (VTE), two investigators independently searched published studies indexed in MEDLINE, EMBASE, and the Cochrane database from inception to April 2016 using the terms for each type of vasculitis in conjunction with the terms for venous thromboembolism. The inclusion criteria were as follows: (1) observational studies published as original studies to evaluate the association between vasculitis and VTE, (2) odds ratios, relative risk or hazard ratio or standardized incidence ratio with 95 % confidence intervals (CI) were provided, and (3) participants without vasculitis were used as comparators for cohort studies and cross-sectional studies while participants without VTE were used as comparators for case-control studies. RevMan 5.3 software was used for the data analysis. Point estimates and standard errors were extracted from individual studies and were combined by the generic inverse variance method of DerSimonian and Laird. Seven studies investigating the risk of VTE among patients with systemic vasculitis were identified. At least one study was available for three specific forms of vasculitis (polyarteritis nodosa [PAN], granulomatosis with polyangiitis [GPA], and giant cell arteritis [GCA]). An increased risk of VTE was seen in all three vasculitides (GPA, pooled RR 3.94, 95 % CI 1.11-14.01; PAN, pooled RR 3.00, 95 % CI 2.20-4.09; GCA, pooled RR 2.26, 95 % CI 1.38-3.71). This meta-analysis demonstrates that patients with systemic vasculitis may have a significantly increased risk of VTE.
Topics: Cross-Sectional Studies; Humans; Incidence; Risk; Vasculitis; Venous Thromboembolism
PubMed: 27572522
DOI: 10.1007/s10067-016-3394-7 -
Annals of Hematology Mar 2016
Review
Topics: Hematopoietic Stem Cell Transplantation; Humans; Male; Middle Aged; Myelodysplastic Syndromes; Polyarteritis Nodosa
PubMed: 26787416
DOI: 10.1007/s00277-015-2587-5 -
International Immunopharmacology Aug 2015Standard therapeutic schemes for vasculitis are usually associated with numerous side effects and uneven clinical response. However, recent advances in understanding of... (Review)
Review
Standard therapeutic schemes for vasculitis are usually associated with numerous side effects and uneven clinical response. However, recent advances in understanding of the pathogenesis of these systemic diseases have resulted in the development of a group of biologic agents potentially useful in patients with vasculitis. Thus, anti-tumor necrosis factor-α drugs may be effective in patients with refractory Kawasaki disease but have failed to do so in giant cell arteritis, and their role in Takayasu arteritis is yet unclear. Preliminary reports on the use of the anti-IL6-receptor antibody, tocilizumab, in large-vessel vasculitis have been encouraging. Interferon alpha has showed positive results in hepatitis B virus-associated polyarteritis nodosa, and hepatitis C virus-induced cryoglobulinemia. Early experience with rituximab in several types of vasculitis has been quite promising, but must be confirmed in ongoing randomized clinical trials. The development of new biologic targeted therapies will probably open a hopeful future for patients with vasculitis.
Topics: Animals; Antibodies, Antineutrophil Cytoplasmic; Biological Therapy; Humans; Systemic Vasculitis
PubMed: 25828585
DOI: 10.1016/j.intimp.2015.03.020