scholarly journals HLA-DR in Brazilian Patients with Polyarteritis Nodosa (PAN) and Microscopic Polyangiitis (MPA)

2009 ◽  
Vol 26 (3) ◽  
pp. 105-109 ◽  
Author(s):  
Alzírton de Lira Freire ◽  
Roseneide Aparecida Conde ◽  
Manoel Barros Bertolo ◽  
Lílian Teresa Lavras Costallat ◽  
Maurício Levy-Neto ◽  
...  
Keyword(s):  
Polyarteritis Nodosa ◽  
Microscopic Polyangiitis ◽  
Systemic Vasculitis ◽  
Severe Disease ◽  
Consensus Conference ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Eular Recommendations ◽  
American College Of Rheumatology ◽  
Hla Dr ◽  
Caucasian Patients

The aim of this study was to evaluate the frequency and clinical associations of HLA-DR alleles in Brazilian Caucasian patients with polyarteritis nodosa (PAN) or microscopic polyangiitis (MPA). We evaluated 29 Caucasian patients with vasculitis classified as PAN or MPA according to the American College of Rheumatology (ACR) 1990 Criteria, Chapel Hill Consensus Conference (CHCC) nomenclature for vasculitis and EULAR recommendations for conducting clinical studies in systemic vasculitis. HLA-DR alleles were typed using polymerase chain reaction-amplified DNA, hybridized with sequence-specific low resolution primers. DNA obtained from 59 Caucasian healthy blood donors were used as control. In order to evaluate if a specific HLA may have influence on the clinical profile of those diseases, we also divided the patients according to Birmingham vasculitis score (BVAS) and Five-Factors Score (FFS) at the time of diagnosis. Increased frequency of HLA-DRB1*16 (p= 0.023) and DRB4*01 (p= 0.048) was found in patients with higher disease activity at the time of diagnosis (BVAS ≥ 22). Patients with less severe disease (FFS = 0) had a higher frequency of HLA-DRB1*03 (p= 0.011). Patients with gastrointestinal tract involvement had significantly increased frequency of HLA-DRB1*11 or B1*12 (p= 0.046), B1*13 (p= 0.021) and B3 (p= 0.008). In contrast, patients with renal disease, had higher frequency of DRB1*15 or DRB1*16 (p= 0.035) and B5 (p= 0.035). In the subgroup of patients with MPA, increased frequency of HLA-DRB1*15 was found in patients with BVAS ≥ 22 (p= 0.038) and FFS ≥ 1 (p= 0.039) suggesting that this allele is associated with more aggressive disease. Antineutrophil cytoplasmic antibodies (ANCA) negative MPA patients had significantly increased frequency of HLA-DRB1*11 or DRB1*12 when compared to ANCA positive patients (p= 0.023). Our results suggest that HLA-DR alleles may influence PAN and MPA clinical expression and outcome and that in MPA they participate in the mechanisms involved in the development to ANCA.

scholarly journals THE RENAL LESION IN SYSTEMIC VASCULITIS

2015 ◽  
pp. 28-33
Author(s):  
O.V. Syniachenko ◽  
T.B. Bevzenko ◽  
M.V. Iermolaieva ◽  
O.O. Diadyk
Keyword(s):  
Immune System ◽  
Kidney Disease ◽  
Rheological Properties ◽  
Polyarteritis Nodosa ◽  
Microscopic Polyangiitis ◽  
Systemic Vasculitis ◽  
Pathological Process ◽  
Renal Lesion ◽  
Cryoglobulinemic Vasculitis ◽  
Churg Strauss

The aim and the objectives: to investigate the frequency and the character of renal lesion in systemic vasculitis, as well as aspects of the pathogenesis of nephropathy in this category of patients. Material and methods: investigate 525 patients, including 193 - with vasculitis Shenlayna-Henoch, 116- with microscopic polyangiitis, 93 - with cryoglobulinemic vasculitis, 39 - with polyarteritis nodosa, 29 - with aortoarteritis Takayasu, 28 - withWegener’s granulomatosis and 27 - with eosinophilic vasculitis Churg-Strauss. Results: the renal lesion is observed in 52-68% cases of vasculitis depends on the age and the sex of the patients, the duration of the disease, the degree of activity of the pathological process, alteration in the heart and lungs, integrated severity extrarenal signs of the disease, at that, the rate ofprogression of nephropathy associated with the character of the disorders of the immune system, the rheological properties of blood and endothelial vessels’function. Conclusions: kidney disease is one of the leading signs of systemic vasculitis and observed in most patients, it is determine the course of the disease.

scholarly journals Atypical Endobronchial Carcinoid with Postobstructive Pneumonia Obscuring the Diagnosis of Granulomatosis with Polyangiitis

2015 ◽  
Vol 2015 ◽  
pp. 1-8
Author(s):  
Robert Ali ◽  
Candice Baldeo ◽  
Jesse Onyenekwe ◽  
Roshan Lala ◽  
Cristian Landa ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Clinical Manifestations ◽  
Granulomatous Inflammation ◽  
Lower Limbs ◽  
Antineutrophil Cytoplasmic Antibodies ◽  
Small Vessel Vasculitis ◽  
American College Of Rheumatology ◽  
Abnormal Chest ◽  
Necrotizing Glomerulonephritis

Granulomatosis with polyangiitis (GPA), previously termed Wegener’s Granulomatosis, is an autoimmune small vessel vasculitis which is highly associated with antineutrophil cytoplasmic antibodies (ANCA) and has varied clinical manifestations. Diagnosis hinges on identifying a combination of clinical features of systemic vasculitis, positive ANCA serology, and histological evidence of necrotizing vasculitis, necrotizing glomerulonephritis, or granulomatous inflammation from a relevant organ biopsy. The American College of Rheumatology has also developed a classification criteria focusing specifically on nasal or oral inflammation, abnormal chest radiograph, and abnormal urinary sediment, along with granulomatous inflammation, which helps to distinguish GPA from other forms of systemic vasculitis. In the case presented below, the diagnosis of GPA was delayed as the patient had a concomitant atypical endobronchial carcinoid which predisposed to postobstructive pneumonia. Fortunately, the papular lesions that developed across her lower limbs prompted further investigations. The return of appropriate serology coincided with progression to alveolar hemorrhage, offering a more complete clinical picture, and when she responded to the combination of steroid, cyclophosphamide, and plasma exchange, the diagnosis of GPA was cinched.

scholarly journals 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Polyarteritis Nodosa

2021 ◽  
Author(s):  
Sharon A. Chung ◽  
Mark Gorelik ◽  
Carol A. Langford ◽  
Mehrdad Maz ◽  
Andy Abril ◽  
...  
Keyword(s):  
Polyarteritis Nodosa ◽  
American College Of Rheumatology

Patients with Rheumatoid Arthritis in Clinical Remission Manifest Persistent Joint Inflammation on Histology and Imaging Studies

2014 ◽  
Vol 41 (11) ◽  
pp. 2153-2160 ◽  
Author(s):  
Allen Anandarajah ◽  
Ralf Thiele ◽  
Ellen Giampoli ◽  
Johnny Monu ◽  
Gwy-Suk Seo ◽  
...  
Keyword(s):  
Rheumatoid Arthritis ◽  
Disease Activity ◽  
Clinical Remission ◽  
Severe Disease ◽  
Joint Inflammation ◽  
Imaging Studies ◽  
American College Of Rheumatology ◽  
Remission Criteria ◽  
Magnetic Resonance Imaging Mri ◽  
Active Inflammation

Objective.The purpose of our study was to test the hypothesis that synovitis on magnetic resonance imaging (MRI) and ultrasound (US) observed in patients with rheumatoid arthritis (RA) who meet remission criteria reflects active inflammation on histopathology.Methods.We analyzed 15 synovial specimens obtained during surgical procedures from 14 patients with RA in clinical remission as defined by the American College of Rheumatology criteria. Histological specimens were scored for hyperplasia of synovial lining and synovial stroma, inflammation, lymphoid follicles, and vascularity. The histology scores were classified as minimal, mild, moderate, or severe disease activity. US and MRI performed within a 4-month period of surgery were scored for disease activity. The correlation between histology and imaging scores was examined.Results.Four of 14 patients were receiving anti-tumor necrosis factor (TNF) therapy, 4 were receiving methotrexate (MTX) alone, 4 were taking MTX and hydroxychloroquine (HCQ), and 1 was taking HCQ and sulfasalazine. Four specimens had severe, 6 moderate, 3 mild, and 2 minimal disease activity on histology. Three of 4 specimens with minimal and mild histology were observed in subjects receiving anti-TNF therapy. Synovitis was noted on greyscale in 80% of joints and Doppler signal in 60%. MRI demonstrated synovitis and bone marrow edema in 86% of images. Positive but not significant correlations were noted between histology and synovitis scores on US.Conclusion.Despite clinical remission, histology and imaging studies documented a persistently active disease state that may explain the mechanism for radiographic progression.

Isolated intestinal polyarteritis nodosa in an elderly patient

2021 ◽  
Vol 14 (3) ◽  
pp. e241431
Author(s):  
Ariana González-Meléndez ◽  
Eduardo J Medina-Parrilla ◽  
Román Vélez ◽  
Luis M Vilá
Keyword(s):  
Abdominal Pain ◽  
Polyarteritis Nodosa ◽  
Elderly Population ◽  
Systemic Vasculitis ◽  
Exploratory Laparotomy ◽  
The Elderly ◽  
High Dose ◽  
Systemic Involvement ◽  
Intestinal Pseudo Obstruction ◽  
Single Organ

Polyarteritis nodosa (PAN) is a necrotising systemic vasculitis involving medium-sized and small-sized vessels. PAN limited to a single organ is rare, particularly in the elderly population. Herein, we present a 73-year-old-woman who developed severe abdominal pain. Mesenteric angiography showed multifocal areas of segmental dilation and narrowing of the superior mesenteric, ileocolic and right colonic arteries. Exploratory laparotomy revealed multiple areas of necrosis of the jejunum for which resection was performed. Histopathological exam disclosed mesenteric vasculitis with fibrinoid necrosis of the arterial wall with leucocytic infiltrates and haemorrhages consistent with PAN. She was started on high-dose corticosteroids with an initial good response. However, 6 months later, she developed intestinal pseudo-obstruction for which oral cyclophosphamide was started. After 5 months of cyclophosphamide therapy, she remained stable without further relapses. Our case suggests that PAN should be considered in elderly patients presenting with abdominal pain even in the absence of systemic involvement.

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