Miscellaneous vasculitides

Author(s):  
Richard A. Watts ◽  
Eleana Ntatsaki

The vasculitides are a group of relatively rare conditions with a broad spectrum of clinical presentations that can cause significant morbidity and mortality. Classification of the vasculitic syndromes is done according to the size of the vessels affected and also the presence of anti-neutrophil cytoplasmic antibodies (ANCA). Vasculitides can be either primary or secondary to an underlying systemic disease, malignancy, or infection. This chapter covers the spectrum of the secondary vasculitides; some of the non-ANCA-associated primary vasculitides and miscellaneous types of vasculitic syndromes. Secondary vasculitis can occur in the background of systemic rheumatic diseases such as rheumatoid arthritis, spondyloarthropathies, or other connective tissue diseases. Vasculitis can also present in relation to precipitants such as drugs (propylthiouracil, hydralazine, leucotriene antagonists) or vaccines. Infection (bacterial, mycobacterial, viral, and fungal) has been associated with vasculitis either as a trigger or as a consequence of iatrogenic immunosuppression. Infection-related vasculitis can affect all types and sizes of vessels. Certain forms of vasculitis such as cryoglobulinaemia are closely associated with viral infections and more specifically with HCV infection. There are forms of vasculitis, which appear to be isolated or localized to a single organ, or site (skin, gastrointestinal, genital, and primary central nervous system vasculitis) that may be histologically similar to systemic syndromes, but have a different prognosis. Other conditions that may mimic vasculitis and miscellaneous conditions such as Cogan’s syndrome and relapsing polychondritis are also discussed.

Author(s):  
Richard A. Watts ◽  
Eleana Ntatsaki

The vasculitides are a group of relatively rare conditions with a broad spectrum of clinical presentations that can cause significant morbidity and mortality. Classification of the vasculitic syndromes is done according to the size of the vessels affected and also the presence of anti-neutrophil cytoplasmic antibodies (ANCA). Vasculitides can be either primary or secondary to an underlying systemic disease, malignancy, or infection. This chapter covers the spectrum of the secondary vasculitides; some of the non-ANCA-associated primary vasculitides and miscellaneous types of vasculitic syndromes. Secondary vasculitis can occur in the background of systemic rheumatic diseases such as rheumatoid arthritis, spondyloarthropathies, or other connective tissue diseases. Vasculitis can also present in relation to precipitants such as drugs (propylthiouracil, hydralazine, leucotriene antagonists) or vaccines. Infection (bacterial, mycobacterial, viral, and fungal) has been associated with vasculitis either as a trigger or as a consequence of iatrogenic immunosuppression. Infection-related vasculitis can affect all types and sizes of vessels. Certain forms of vasculitis such as cryoglobulinaemia are closely associated with viral infections and more specifically with HCV infection. There are forms of vasculitis, which appear to be isolated or localized to a single organ, or site (skin, gastrointestinal, genital, and primary central nervous system vasculitis) that may be histologically similar to systemic syndromes, but have a different prognosis. Other conditions that may mimic vasculitis and miscellaneous conditions such as Cogan's syndrome and relapsing polychondritis are also discussed.


2006 ◽  
Vol 104 (1) ◽  
pp. 143-146 ◽  
Author(s):  
Kazufumi Sato ◽  
Toshihiko Kubota ◽  
Ryuhei Kitai ◽  
Isamu Miyamori

✓ Relapsing polychondritis (RP) is a rare systemic disease characterized by recurrent inflammation of the cartilaginous structures and connective tissue. Central nervous system lesions in association with RP have occasionally been reported, but intracranial mass lesions have not been described. The authors report the first such case, in which a 51- year-old man presented with parasagittal meningeal plasma cell granuloma with RP. The mass was subtotally resected and adjuvant radiotherapy was administered. The patient did not experience any recurrence of the lesion during an 8-year follow-up period. In this case, the exact diagnosis of RP was made based on symptoms of respiratory tract chondritis, which was successfully treated by the placement of tracheobronchial stents.


2001 ◽  
Vol 47 (6) ◽  
pp. 1089-1093 ◽  
Author(s):  
Nicola Bizzaro ◽  
Giovanni Mazzanti ◽  
Elio Tonutti ◽  
Danilo Villalta ◽  
Renato Tozzoli

Abstract Background: Rheumatoid arthritis (RA) is the most common autoimmune rheumatic disease, but specific and practicable tests for its diagnosis are lacking. We evaluated the diagnostic accuracy of a new commercial ELISA in detecting anti-cyclic citrullinated peptide (CCP) antibodies for the diagnosis of RA. Methods: Anti-CCP antibodies were determined in 330 serum samples: 98 from RA patients and 232 from controls, including patients with connective tissue diseases, other rheumatic diseases, viral infections, Lyme disease, autoimmune thyroiditis, cancer, and monoclonal gammopathy, and sex- and age-matched healthy subjects. Intra- and interassay CVs were 5–13% and 9–17%, respectively. Rheumatoid factor (RF) was also assayed in every sample, and results were compared to anti-CCP for sensitivity and specificity. Results: At a cutoff value of 50 units, sensitivity was 41% (confidence interval, 31–50%) and specificity was 97.8% (95–100%). Anti-CCP-positive RA patients had a mean antibody concentration of 1100 units (range, 57–3419 units), and anti-CCP-negative RA patients and controls had mean values of 7.6 and 6.8 units, respectively (range, 1–39 units). The area under the ROC curve was 0.71 (95% confidence interval, 0.63–0.78). RF had a higher sensitivity (62%) and a lower specificity (84%) than anti-CCP. When the two antibodies were used together, specificity was 99.6%. Conclusion: Anti-CCP antibody testing may be useful if performed concomitantly with RF assay to diagnose patients with suspected early RA.


2018 ◽  
Vol 31 (4) ◽  
pp. 209-212
Author(s):  
Daria Bednarek-Hatlinska ◽  
Anna Prymas ◽  
Marta Mrall-Wechta ◽  
Anna Surdacka

Abstract Dentistry, is one of the intensively and rapidly growing branches of medicine. This prompts dentists to take an interdisciplinary approach to their patients. Thus, the dentist, being a general practitioner, can make significant contributions to the early diagnosis of systemic disease and the faster implementation of appropriate treatment. In view of the aforementioned, we undertook research on the relationship of pathological changes observed in the oral cavity with diseases of the connective tissue system. Collagenosis is a chronic autoimmune disease initiated by many factors, among which the genetic factor and viral infections are mentioned. The changes observed in the oral cavity may be a picture of the disease, a complication of the disease or a side effect of the treatment. The aim of the study is, thus, too present the pathological changes in the oral cavity which often accompany collagenosis, and to discuss the risk factors of connective tissue system diseases and methods of dental treatment.


2011 ◽  
Vol 1 ◽  
pp. 32 ◽  
Author(s):  
Harbir S Sidhu ◽  
Gauraang Bhatnagar ◽  
Pervinder Bhogal ◽  
Richard Riordan

Rheumatoid arthritis (RA) is a common disorder that affects the joints. RA is a systemic disease associated with relatively frequent and variable pleuropulmonary manifestations. This article reviews the common and potentially serious thoracic sequelae in terms of pleural disease, pulmonary nodules, airways disorders, and interstitial disease, as well as pulmonary side effects of antirheumatic medication. An imaging-guided approach to classification of RA-associated lung disease is outlined and the comparative values of different imaging modalities are discussed. An appreciation of current knowledge of epidemiology, pathological correlation, and prognostic implications of different RA-associated lung disease is provided. We highlight importance of considering pertinent differential diagnoses to avoid misdiagnosis, and outline common pitfalls in dealing with pleuropulmonary rheumatoid disease.


1996 ◽  
Vol 35 (04/05) ◽  
pp. 334-342 ◽  
Author(s):  
K.-P. Adlassnig ◽  
G. Kolarz ◽  
H. Leitich

Abstract:In 1987, the American Rheumatism Association issued a set of criteria for the classification of rheumatoid arthritis (RA) to provide a uniform definition of RA patients. Fuzzy set theory and fuzzy logic were used to transform this set of criteria into a diagnostic tool that offers diagnoses at different levels of confidence: a definite level, which was consistent with the original criteria definition, as well as several possible and superdefinite levels. Two fuzzy models and a reference model which provided results at a definite level only were applied to 292 clinical cases from a hospital for rheumatic diseases. At the definite level, all models yielded a sensitivity rate of 72.6% and a specificity rate of 87.0%. Sensitivity and specificity rates at the possible levels ranged from 73.3% to 85.6% and from 83.6% to 87.0%. At the superdefinite levels, sensitivity rates ranged from 39.0% to 63.7% and specificity rates from 90.4% to 95.2%. Fuzzy techniques were helpful to add flexibility to preexisting diagnostic criteria in order to obtain diagnoses at the desired level of confidence.


Author(s):  
Abeer Fauzi Al-Rubaye ◽  
Mohanad Jawad Kadhim ◽  
Imad Hadi Hameed

The pharmacological mechanisms of the medicinal plants traditionally used for RA in Persian medicine are discussed in the current review. Further investigations are mandatory to focus on bioefficacy of these phytochemicals for finding novel natural drugs. Rheumatoid arthritis is chronic, progressive, disabling autoimmune disease characterized by systemic inflammation of joints, damaging cartilage and bone around the joints. It is a systemic disease which means that it can affect the whole body and internal organs such as lungs, heart and eyes. Although numbers of synthetic drugs are being used as standard treatment for rheumatoid arthritis but they have adverse effect that can compromise the therapeutic treatment. Unfortunately, there is still no effective known medicinal treatment that cures rheumatoid arthritis as the modern medicine can only treat the symptoms of this disease that means to relieve pain and inflammation of joints. It is possible to use the herbs and plants in various forms in order to relieve the pain and inflammation in the joints. There are so many medicinal plants that have shown anti rheumatoid arthritis properties. So the plants and plant product with significant advantages are used for the treatment of rheumatoid arthritis. The present review is focused on the medicinal plants having anti rheumatoid arthritis activity


Author(s):  
Andrew M. Ferry ◽  
Rami P. Dibbs ◽  
Shayan M. Sarrami ◽  
Amjed Abu-Ghname ◽  
Han Zhuang Beh ◽  
...  

AbstractCraniofacial surgery in children is a highly challenging discipline that requires extensive knowledge of craniofacial anatomy and pathology. Insults to the fronto-orbital skeleton have the potential to inflict significant morbidity and even mortality in patients due to its proximity to the central nervous system. In addition, significant aesthetic and ophthalmologic disturbances frequently accompany these insults. Craniosynostosis, facial trauma, and craniofacial tumors are all pathologies that frequently affect the fronto-orbital region of the craniofacial skeleton in children. While the mechanisms of these pathologies vary greatly, the underlying principles of reconstruction remain the same. Despite the limited data in certain areas of fronto-orbital reconstruction in children, significant innovations have greatly improved its safety and efficacy. It is imperative that further investigations of fronto-orbital reconstruction are undertaken so that craniofacial surgeons may provide optimal care for these patients.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 560.3-561
Author(s):  
E. F. Vicente-Rabaneda ◽  
J. De la Macorra ◽  
J. P. Baldivieso ◽  
F. Gutiérrez-Rodríguez ◽  
A. García-Vadillo ◽  
...  

Background:Interstitial lung disease (ILD) is a severe manifestation of rheumatoid arthritis (RA), linked to increased mortality. There is still no consensus on the best therapeutic strategy as there aren’t yet randomized controlled trials.Objectives:To analyze the available scientific evidence on the efficacy and safety of rituximab (RTX) treatment of interstitial lung disease (ILD) associated with rheumatoid arthritis (RA).Methods:A systematic search was carried out in PubMed until April 2020 following the PRISMA recommendations. Studies were selected according to the following inclusion criteria: (1) original research, including case series, case/control studies, cohort studies, and clinical trials; (2) population with RA and associated ILD, either monographically or together with other connective tissue diseases (CTD), provided that individualized data on patients with RA were provided; (3) patients treated with RTX; (4) objective and quantifiable results on the evolution of ILD after treatment with available data of FVC, DLCO and/or HRCT.Results:Of the 64 papers identified, 9 articles were selected. The studies showed great heterogeneity in design, both in the sample selection criteria and in the objectives of the analysis. Most were observational, retrospective (n = 6) or prospective (n = 2) studies, with only one open prospective experimental study. Those focused on RA predominated, but 3 of them also included patients with other CTDs. The mean age of the patients in the different studies ranged between 52 and 70 years, predominantly women. 40-79% had a history of smoking and were mostly positive for rheumatoid factor (83-100%) and anti-CCP (82-100%). The most frequent radiological patterns were NSIP, UIP and undefined. The outcome measures were diverse: changes in respiratory function tests (LTF) and HRCT, incidence of pulmonary dysfunction, mortality rates, effect on glucocorticoid deprivation, delay in inclusion in the lung transplant list and/or serious adverse events. The initiation of RTX was motivated by pulmonary and/or joint pathology, in patients with failure to other synthetic or biological DMARDs. A total of 393 treatment cycles were collected in 114 patients, with a mean of 3.45 cycles per patient. The RTX regimen was 2 infusions of 1g 2 weeks apart in all patients, except for 1 who received the lymphoma-like regimen. With regard to the efficacy of the treatment with RTX, improvement and especially stabilization of HRCT and LFT predominated, with numerically greater improvement for DLCO than for FVC. There was also a favorable trend in the evolution of patients treated with RTX compared to controls, although it did not reach statistical significance, and a lower risk of deterioration of lung function in patients treated with RTX versus those who had received other DMARDs. The mortality rate found at 5 years was lower than that previously described for the disease and half for the patients treated with RTX compared to those treated with anti-TNF. The adverse events described in the studies did not show additional safety alerts to those already described for RTX.Conclusion:RTX seems to be postulated as a promising therapy for patients with ILD associated with RA, showing a stabilizing effect on the lung function, with an acceptable safety profile. However, further research of higher methodological quality prospective studies is needed to confirm these favorable preliminary results.Disclosure of Interests:None declared


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