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State of the art in the treatment of systemic vasculitides.

ABSTRACT: Anti-neutrophil cytoplasm antibodies (ANCA) are associated with small vessel vasculitides (AASV) affecting the lungs and kidneys. Structured clinical assessment using the Birmingham Vasculitis Activity Score and Vasculitis Damage Index should form the basis of a treatment plan and be used to document progress, including relapse. Severe disease with organ or life threatening manifestations needs cyclophosphamide or rituximab, plus high dose glucocorticoids, followed by lower dose steroid plus azathioprine, or methotrexate. Additional plasmapheresis is effective for very severe disease, reducing dialysis dependence from 60 to 40% in the first year, but with no effect on mortality or long-term renal function, probably due to established renal damage. In milder forms of ANCA-associated vasculitis, methotrexate, leflunomide, or mycophenolate mofetil are effective. Mortality depends on initial severity: 25% in patients with renal failure or severe lung hemorrhage; 6% for generalized non-life threatening AASV but rising to 30-40% at 5?years. Mortality from GPA is four times higher than the background population. Early deaths are due to active vasculitis and infection. Subsequent deaths are more often due to cardiovascular events, infection, and cancer. We need to improve the long-term outcome, by controlling disease activity but also preventing damage and drug toxicity. By contrast, in large vessel vasculitis where mortality is much less but morbidity potentially greater, such as giant cell arteritis (GCA) and Takayasu arteritis, therapeutic options are limited. High dose glucocorticoid results in significant toxicity in over 80%. Advances in understanding the biology of the vasculitides are improving therapies. Novel, mechanism based therapies such as rituximab in AASV, mepolizumab in eosinophilic granulomatosis with polyangiitis, and tocilizumab in GCA, but the lack of reliable biomarkers remains a challenge to progress in these chronic relapsing diseases.

SUBMITTER: Luqmani RA

PROVIDER: S-EPMC4195356 | biostudies-literature | 2014

REPOSITORIES: biostudies-literature

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State of the art in the treatment of systemic vasculitides.

Luqmani Raashid Ahmed RA

Frontiers in immunology 20141013

Anti-neutrophil cytoplasm antibodies (ANCA) are associated with small vessel vasculitides (AASV) affecting the lungs and kidneys. Structured clinical assessment using the Birmingham Vasculitis Activity Score and Vasculitis Damage Index should form the basis of a treatment plan and be used to document progress, including relapse. Severe disease with organ or life threatening manifestations needs cyclophosphamide or rituximab, plus high dose glucocorticoids, followed by lower dose steroid plus aza ...[more]

PMID: 25352843

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Project description:Systemic necrotizing vasculitis comprises a group of diseases resembling polyarteritis nodosa and anti-neutrophil cytoplasmic antibody-associated vasculitis (ANCA): granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and microscopic polyangiitis. The definitive diagnosis is made in cooperation with a reference center for autoimmune diseases and rare systemic diseases or a competency center. The management goals are: to obtain remission and, in the long term, healing; to reduce the risk of relapses; to limit and reduce the sequelae linked to the disease; to limit the side effects and the sequelae linked to the treatments; to improve or at least maintain the best possible quality of life; and to maintain socio-professional integration and/or allow a rapid return to school and/or professional activity. Information and therapeutic education of the patients and those around them are an integral part of the care. All health professionals and patients should be informed of the existence of patient associations. The treatment of vasculitis is based on variable combinations of glucocorticoids and immunosuppressants, chosen and adapted according to the disease concerned, the severity and/or extent of the disease, and the underlying factors (age, kidney function, etc.). Follow-up clinical and paraclinical examinations must be carried out regularly to clarify the progression of the disease, detect and manage treatment failures and possible relapses early on, and limit sequelae and complications (early then late) related to the disease or treatment. A distinction is made between the induction therapy, lasting approximately 3-6 months and aimed at putting the disease into remission, and the maintenance treatment, lasting 12-48 months, or even longer. The role of the increase or testing positive again for ANCA as a predictor of a relapse, which has long been controversial, now seems to have greater consensus: Anti-myeloperoxidase ANCAs are less often associated with a relapse of vasculitis than anti-PR3 ANCA.

Project description:IntroductionBreast edema can arise from different etiologies; however, it is mostly seen after breast conserving surgery and/or radiotherapy. Combining breast conserving surgery and radiotherapy can cause damage to the lymphatic system and reactions to surrounding tissues, which can lead to breast edema; hereby, the breast size can increase by more than one cup size. Swelling of the breast is not the only criterion associated with breast edema. Other common criteria found in literature are peau d'orange, heaviness of the breast, skin thickening, breast pain, redness of the skin, hyperpigmented skin pores and a positive pitting sign. Despite the benefits of breast conserving surgery, breast edema can be uncomfortable, and can negatively influence quality of life in suffering patients. In contrast to lymphedema of the arm, which is well known in clinical practice and in research, breast edema is often underestimated and far less explored in literature. Currently, many aspects still need to be reviewed.Purpose and importance to practiceThis masterclass aims at providing the state of the art of breast edema for all health care workers and researchers involved in the treatment and monitoring of breast cancer patients. It includes current and future perspectives on its diagnosis, longitudinal course and treatment. Furthermore, recommendations for clinical practice and future research are discussed.Clinical implicationsIt is recommended to closely monitor those patients in whom breast edema symptoms do not decline within 6 months after termination of radiotherapy and provide them with the appropriate therapy. Since evidence concerning the treatment of breast edema is currently lacking, we recommend the complex decongestive therapy (CDT) to the utmost extent, by analogy with the lymphedema treatment of the extremities. This treatment involves skin care, exercise therapy and compression. Additionally, all patients should be informed about the normal course of breast edema development.Future research prioritiesA consensus should be reached among clinicians and researchers concerning the definition, assessment methods and best treatment of breast edema. Furthermore, high quality studies are necessary to prove the effectiveness of the CDT for breast edema.

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State of the art in the treatment of systemic vasculitides.

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State of the art in the treatment of systemic vasculitides.

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