Vasculitis: After the Diagnosis

Vasculitis Patient Management

While it is the findings of a detailed patient history, serological testing, and clinical observation that help inform a diagnosis of ANCA-associated vasculitides, the management of these diseases will be driven largely by the patient’s signs, symptoms, and personal risk factors.

ANCA vasculitides are rare and difficult to diagnose, but doing so is imperative. They are diseases characterized by considerable mortality and morbidity. Early diagnosis has the capacity to produce a more favorable long-term outcome for patients with these serious diseases. Recommended treatment of ANCA-associated vasculitides can be divided into two phases—initial treatment for rapid onset of remission and subsequent maintenance therapy to prevent relapse.¹

Several well-established clinical guidelines can help facilitate the creation of a management plan that can then be personalized to meet that patient’s needs:

Prognosis

It is generally accepted that patients with ANCA-associated vasculitides have a very poor, if not severe, prognosis when these diseases are not diagnosed, evaluated, and subsequently treated swiftly and correctly.² Relapse of ANCA-associated vasculitides are common, and studies have shown that up to 50% of diagnosed patients will have returning signs and symptoms.¹ These diseases carry with it an increased risk for cardiovascular-related deaths,² providing another reason for close, personalized monitoring. Those over the age of 70 should also be closely monitored, as they face an increase in mortality compared with younger patients.²

Prognosis of ANCA-associated vasculitides are contingent upon the specific disease from which your patient is suffering.

Granulomatosis with polyangitis:

A variety of factors can influence a patient’s prognosis: early diagnosis, when treatment is started, the response to treatment, and how severely the individual is affected, can all contribute to patient outcomes. With appropriate treatment, the outlook is generally fair; though survival rates decline with increased age, increased serum creatinine concentration and 24-h urinary protein dialysis dependence.³

Eosinophilic granulomatosis with polyangiitis (Churg Strauss syndrome):

The long-term prognosis for people with eosinophilic granulomatosis with polyangiitis has improved significantly since the use of certain therapies for people with more severe disease has been employed. Most reports now suggest a survival rate of 70 to 90 percent at five years.⁴

Microscopic polyangiitis:

Early diagnosis, coupled with prompt treatment, can lead to an improved prognosis in patients with microscopic polyangiitis. With treatment, 75% of individuals may achieve complete remission.⁵

Prognosis is generally considered poor in patients with ANCA-associated vasculitides, as patients may sustain permanent damage to their kidneys, lungs and respiratory tract, heart, peripheral and central nervous system, along with total or partial loss of sight or hearing. Severe fatigue, muscle weakness and chronic pain are frequent, direct consequences of ANCA-associated vasculitides. Side effects of treatment can be serious.

Comobridities

ANCA-associated vasculitides are rare, but there are indicators that can alert you to its potential presence. Certain drugs, for example, may contribute to the onset of ANCA-associated vasculitides. Studies have shown that long term treatment of autoimmune thyroid diseases, in the form of antithyroid drug treatment, is a frequent trigger of ANCA-associated autoimmune diseases.⁶

Common comorbidities seen include:⁷

Osteoporosis (rate ratio 4.6, 95% CI 3.0–7.0),
Venous thromboembolism (4.0, 95% CI 1.9–8.3),
Thyroid diseases (2.1, 95% CI 1.3–3.3),
Diabetes mellitus (2.0, 95% CI 1.3–2.9)

Being able to quickly and correctly identify ANCA-associated vasculitides can mean the difference in the success of a patient’s outcome.

Know the signs and symptoms of autoimmune thyroid diseases that could potentially trigger vasculitis.

Thyroid Disease Overview >

Precise Management - An Approach Unique to Each Patient

Once a patient has been diagnosed with ANCA-associated vasculitides, time is of the essence. A tailored treatment plan, depending on the presence of comorbidities, personal risk factors, clinical manifestations, and the patient’s demographics, is an important ingredient to successful management. Established guidelines for the clinical care of ANCA-associated vasculitides patients usually involves potent immunosuppressive drugs, which often carry a risk of significant side effects.⁸

Though the immediate survival rate for ANCA-associated vasculitides has improved, the real goals of long-term management should be centered around reducing the relapse rate, improving renal survival, and reducing the development of cardiovascular disease and malignancies. Periodic follow up assessment of cardiovascular risk in patients diagnosed with ANCA-associated vasculitides is recommended.⁸ Ongoing, long-term follow-up care can help inform modifications needed to the treatment plan and help get you and your patient closer to these treatment goals.

Continued Testing and Monitoring

Careful long-term management with periodic follow ups may preserve patient health and improve outcomes. Maintenance of remission in ANCA-associated vasculitides requires long-term therapy in most patients.¹

It is recommended that patients suffering from ANCA-associated vasculitides are given a clear verbal explanation of the nature of their disease, the treatment options, the side effects of treatment, and the short-term and long-term prognosis, as this is associated with improved patient outcomes.⁸

As a part of ongoing care, you may continue to monitor for overlapping symptoms and clinical events. Several tests exist that can help you do so, the results of which can also help rule in or out the existence of other autoimmune disease that may complicate the prognosis and treatment plan.

Could your patient with ANCA-associated vasculitides be at risk for more than one disease?

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Conditions & Diseases

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Tests

Diagnostic tests give reliable results that support primary care physicians as well as specialists in providing optimal patient management.