Diagnosis Of Primary Vasculitis Research Articles

Leprosy mimicking ANCA-associated vasculitis

Vasculitis represents a large group of diseases, classified between primary and secondary. Diagnosis of primary vasculitis is a challenge in medical practice, since there are wide and heterogeneous clinical manifestations, with diagnostic criteria still scarce. Some clinical manifestations are common: constitutional status, myalgia, arthralgia, arthritis, papules, nodules and ulcers. One of the clinical forms is the limited one in which patients with upper respiratory tract involvement often evolve with systemic disease. Most patients have nasal, sinus or ear involvement that may be present weeks or months before other symptoms. Secondary vasculitis can be related to infections, drugs, toxic substances and neoplasms. The virchowian and dimorphic form of leprosy has similar clinical and serological characteristics with rheumatological diseases. As it is an endemic disease in Brazil, there is a description of a wide variety of clinical presentations, so making a differential diagnosis is essential.

Novel method using DW-MRI and ADC images to guide stereotactic biopsy for the diagnosis small primary angiitis of the central nervous system: a case report

ObjectiveTo determine the role of diffusion-weighted magnetic resonance imaging (DW-MRI) and apparent diffusion coefficient (ADC) imaging to guide stereotactic biopsy for the diagnosis of intracranial angiitis.Case presentationIn a 28-year-old woman who had experienced inactive headache and right limbs numbness for 4 days, preoperative magnetic resonance (MR) scanning, enhanced scanning, diffusion tensor imaging, magnetic resonance spectroscopy, diffusion-weighted imaging (DWI), and ADC image scanning were performed. Stereotactic biopsy was performed in one target where the area of edema detected with MR FLAIR, and two targets where the area shown as a high-value and a lower value area in the DWI/ADC image. Pathological examinations together with computed tomographic and enhanced MRI scans were conducted after surgery. A preoperative enhanced MRI scan showed a uniform low-intensity lesion in the patient’s left centrum semiovale, with a volume of 3.1 cm3. The DWI and ADC images showed uneven high-intensity signals and different ADC values in the lesion area, respectively. During surgery, tissues around the lesion and the lesion center were sampled at the three selected targets. The postoperative pathological diagnosis was primary angiitis of the central nervous system, and the patient was given anti-inflammatory medication and hormone therapy. The 3-year follow-up confirmed that the patient had recovered well, with a Glasgow Outcome Scale score of five.ConclusionDW-MRI and ADC images can be reliably used to determine the location of small intracranial lesions, and guide stereotactic biopsy to facilitate the diagnosis of primary vasculitis of the central nervous system.

Practical approach to vasculitides in adults: an overview of clinical conditions that can mimic vasculitides closely

Primary systemic vasculitides are rare diseases affecting blood vessel walls. The type and patterns of distribution of the organs affected usually reflect the size of the vessels predominantly involved, and the patterns of clinical manifestations are generally useful to reach a specific diagnosis. However, presenting symptoms may lack adequate specificity for a prompt diagnosis, leading to a diagnostic (and therapeutic) delay, often causing irreversible damage to the affected organs. Due to their rarity and variable clinical presentation, the diagnosis of primary vasculitides could be challenging for physicians. Vasculitis mimickers, i.e. the clinical conditions that could be likely mistaken for vasculitides, need to be carefully ruled out, especially before starting the immunosuppressive therapy. We present here a practical approach to the diagnosis of primary systemic vasculitides involving large, medium and small size vessels, and reviewed most of the conditions that could mimic primary systemic vasculitides.

Algorithm for diagnosis of primary vasculitides.

Primary vasculitides are diseases with a wide variety of anatomical, clinical, radiological, and laboratory presentations. Primary vasculitides are difficult to diagnose because of the complexity of clinical presentation, which may lead to delayed treatment and increased financial costs of workup investigations involving non-essential tests. Our objective in the present study is to create an algorithm that helps diagnosis of Primary vasculitides. The algorithm presented in this article allows fast, simple and cost-effective diagnosis of primary vasculitides using just clinical concepts and a few laboratory tests.

Anti‐carbonic anhydrase III autoantibodies in vasculitis syndrome

To identify autoantibodies useful in the diagnosis of primary vasculitides. The presence of antibodies against proteins in the lysate of mouse blood vessels was examined by two-dimensional electrophoresis followed by Western blotting for the pooled serum sample from patients with various forms of vasculitis: polyarteritis nodosa (PAN), microscopic polyangiitis (MPA), Wegener's granulomatosis (WG) and Takayasu's arteritis (TA). Autoantigenicity in patients with vasculitides was examined by Western blotting and enzyme-linked immunosorbent assay (ELISA). Clinicopathological correlations between the positivity of the autoantibodies and clinical status of patients with the vasculitis were examined. The autoantigen detected in the lysate of pooled sera from patients with vasculitides was identified by mass spectrometry as carbonic anhydrase III (CAIII). ELISA showed significantly higher prevalence of anti-CAIII antibodies in MPA patients (MPA, 11/23 [47.8%]; healthy controls, 2/32 [6.3%]; P < 0.001). Further, anti-CAIII antibody-positive MPA patients had higher vasculitis activity scores compared to anti-CAIII antibody-negative patients, and a weak and not significant negative correlation was observed between anti-CAIII antibody levels and myeloperoxidase - anti-nuclear cytoplasmic antibody (MPO-ANCA) levels. No significant differences were found in anti-CAIII autoantibody levels between MPA and the other primary vasculitides. We found significantly high prevalence of anti-CAIII antibody levels in sera from MPA patients. Although the number of samples available in this study is small and anti-CAIII autoantibodies display weak specificity for MPA, anti-CAIII antibodies may be useful for diagnosing MPA in patients who have no ANCA, as well as for assessing disease activity.