The Recommendations for the Management of ANCA-Associated Vasculitis(Part 2)
Before any discussions, this column wants to clarify that the statements here are called recommendations as opposed to ‘guidelines’ or ‘points to consider’ because they offer guidance which needs to be tailored to meet individual needs.
The recommendations are for the healthcare professionals in specialist training and for students of medicine, pharmaceutical industries and drug regulatory agencies.
Experts recommend that patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) have been updated (AAV) are managed in close collaboration with, or at healthcare facilities.
A positive biopsy is strongly supportive of a diagnosis of vasculitis and experts recommend biopsies to assist to come up with a new diagnosis and for further scrutiny for patients suspected of having relapsing vasculitis.
In the case of emission-induction of new-onset organ-threatening or life-threatening AAV, the top experts recommend treatment with a combination of glucocorticoids and either cyciophosphamide or rituximab.
Furthermore, in the case of remission-induction of non organ-threatening AAV, the recommendation is for treatment with a combination ofglucocorticoids and either methotrexate or mycophenolate mofetil.
In the case of a major relapse of organ-threatening or life-threatening disease in AAV the recommended treatment is a combination of glucocaorticoids and either cyclophosphamide or rituximab.
Plasma exchange should be eyed for patients with AAV and a serum creatine level of >500 pmol/L(5.7 mg/dL) due to rapidly progressive giomerulanephritis in the setting of new or relapsing disease.
Plasma exchange may be considered for the treatment of severe diffuse alveolar hemorrhage.
In cases of remission-maintenance of AAV, the recommendation is for a treatment with a combination of low-dose glucocorticoids and either azathioprine, rituximab, methotrexate or mycophenolate mofetil.
Experts recommend that remission-maintenance therapy fur AAV be cantinued for at least 24 months following induction of sustained remission.
In patients with AAV refractory to remission-induction therapy; the recommended treatment is to switch from cyclophosphamide to rituximab or from rituximab to cycophosphamide.
The doctors need to closely managed and when needed, referred to a expert center and be considered for clinical trials.
The experts recomrnend that structured clinical assesment rather than ANCA testing should inform decisions on changes in treatment for AAV.
Doctors should probe the persistent unexplained hematuria in patiernts with prior exposure to cydlaphosphamide.
Hypoimmunoglobuilinaemia has been seen after treatment with rituximab. In this case, the recommendation is for the testing of serum immunoglobulin levels prior to each course of rituximab and in patients with recurrent infection.
Experts are recommending periodic assessment of cardiovascular risk for patients with AAV.
It is recommended that patients with AAV should be given a dear verbal explanation of the nature of their disease the treatment options, the side effects of treatment, and the short-term and long-term prognoses.
Following the remission-induction phase of treatment, it is recommended that patients with AAV should be assessed for the extent and ongoing impact of comorbidities associated with their diagnosis.
AAV is a very variable disease group which is unpredictable and potentially may end up in premature death.
AAV treatment often involves potent immunosuppressive drugs, often with risk of significant side effects.
It is possible to achieve remission without the need of drugs, but a relapse is common. AAV adversely affects quality of life even in patients thought to have clinical remission. This may be an effect of the disease or its treatment.
Next week we will continue our discussions with other recommendations that will help in the management of AAV.