Adalimumab key unlocks blood vessel, dissolving blockades to promote healing.

Hope for Vasculitis: How Adalimumab Offers a New Path to Remission

"Exploring the potential of Adalimumab in treating Hepatitis B virus-negative polyarteritis nodosa, offering renewed hope for patients struggling with disease relapse and treatment failure."


Polyarteritis nodosa (PAN) presents a significant challenge in rheumatology due to its rarity and complexity. This primary systemic vasculitis (PSV) primarily affects medium- or small-sized arteries, distinguishing itself by its lack of association with anti-neutrophil cytoplasmic antibodies (ANCA). Historically, PAN's natural progression led to severe outcomes; however, the introduction of corticosteroids marked a turning point, significantly improving survival rates.

Current therapeutic strategies for PAN are stratified by the Five-Factor Score (FFS), a prognostic tool developed by the French Vasculitis Study Group. Patients without FFS typically undergo treatment with corticosteroids alone, whereas those with critical organ involvement—including renal, gastrointestinal, and cardiac manifestations—receive a combination of corticosteroids and cyclophosphamide. In cases where these standard treatments fail or the disease relapses, biologic agents are considered.

This article examines the potential of adalimumab (ADA), a TNF monoclonal antibody (mAb), in managing PAN, particularly in patients experiencing relapse. By sharing the findings of a case study, we shed light on how ADA can induce clinical remission and prevent disease flares, offering a promising outlook for individuals grappling with this challenging condition.

Adalimumab: A Ray of Hope for Relapsing Vasculitis?

Adalimumab key unlocks blood vessel, dissolving blockades to promote healing.

In August 2012, a 42-year-old Han Chinese male sought medical attention at the dermatology clinic of National Cheng Kung University Hospital, presenting with a six-month history of purpuric reticulate erythema and nodules on his lower extremities. A nodule biopsy revealed dense infiltrates composed of abundant neutrophils and lymphocytes surrounding a subcutaneous small artery, accompanied by neutrophilic fibrin-platelet thrombus in the lumen, consistent with the histopathological characteristics of PAN.

The patient's medical history included weight loss, diffuse myalgia, ankle arthralgia, and testicular pain, leading to a referral to the rheumatology clinic. Physical examinations revealed swollen ankle joints and an enlarged, indurated right testis. Laboratory tests showed elevated ESR and CRP levels, normal hemogram data, normal creatinine levels, and no significant urinalysis abnormalities. Further tests ruled out autoantibodies, ANCA, antinuclear antibody, lupus anticoagulant, and rheumatoid factor. Hepatitis B and C viral markers were also negative, and imaging studies showed no abnormalities.

  • Initial Treatment: The patient was initially diagnosed with PAN based on the 2012 updated Chapel Hill consensus definitions and treated with high-dose corticosteroids (1 mg/kg/day prednisolone).
  • Maintenance Therapy: Upon achieving clinical remission, the prednisolone dosage was gradually reduced and replaced with azathioprine (2 mg/kg/day).
  • Relapse: Despite maintenance therapy, the patient experienced a relapse, characterized by recurrent weight loss, right ankle swelling, right testicular pain, and new-onset hypertension.
  • Adalimumab Intervention: Due to the patient's preference against corticosteroids and their side effects, adalimumab (ADA) 40mg biweekly subcutaneous injection was prescribed without other immunosuppressive agents from October 2014 to December 2016.
  • Outcomes: The ADA treatment resulted in the absence of ankle swelling and testicular pain, with no disease flares or infection complications during the 26-month treatment period.
Upon discontinuation of ADA due to financial constraints, the patient experienced a recurrence of symptoms, including bilateral ankle swelling, nodular lesions on the left leg, and severe aching pain in the right leg and foot, confirmed as peroneal and tibial neuropathy. The patient was then restarted on high-dose corticosteroids, with improvement in arthritis and skin lesions. The addition of weekly methotrexate is being considered to manage his PAN-associated neuropathy.

A Promising Step Forward

This case study illuminates the potential of adalimumab as an effective treatment for PAN, especially in cases of relapse. While further research through large-scale randomized trials is necessary, these findings offer a beacon of hope for individuals grappling with this difficult-to-treat condition.

About this Article -

This article was crafted using a human-AI hybrid and collaborative approach. AI assisted our team with initial drafting, research insights, identifying key questions, and image generation. Our human editors guided topic selection, defined the angle, structured the content, ensured factual accuracy and relevance, refined the tone, and conducted thorough editing to deliver helpful, high-quality information.See our About page for more information.

Everything You Need To Know

1

What is polyarteritis nodosa (PAN), and why is it important to understand this condition?

Polyarteritis nodosa (PAN) is a rare and complex primary systemic vasculitis (PSV) that affects medium- and small-sized arteries. It is characterized by the absence of anti-neutrophil cytoplasmic antibodies (ANCA). Its significance lies in its potential to cause severe outcomes if untreated, affecting various organs and leading to complications like renal, gastrointestinal, and cardiac issues. Timely diagnosis and appropriate treatment are crucial to prevent disease progression and improve patient outcomes.

2

What is the Five-Factor Score (FFS), and how does it influence the treatment approach for polyarteritis nodosa (PAN)?

The Five-Factor Score (FFS) is a prognostic tool used to stratify patients with polyarteritis nodosa (PAN). Its importance lies in guiding treatment decisions. Patients without FFS typically receive corticosteroids alone, while those with critical organ involvement receive a combination of corticosteroids and cyclophosphamide. This scoring system helps tailor treatment strategies based on disease severity and potential risks.

3

What is adalimumab (ADA), and what role might it play in the treatment of polyarteritis nodosa (PAN)?

Adalimumab (ADA) is a TNF monoclonal antibody (mAb) that has shown promise in managing polyarteritis nodosa (PAN), especially in cases of relapse or when standard treatments like corticosteroids and cyclophosphamide fail. Its significance lies in its potential to induce clinical remission and prevent disease flares, offering a valuable alternative for individuals struggling with this challenging condition. By targeting TNF, adalimumab helps reduce inflammation and control the disease.

4

Why did the patient want to avoid corticosteroids and what are the side effects?

Corticosteroids are a class of medications used to treat polyarteritis nodosa (PAN) that work by reducing inflammation in the body. These drugs can have significant side effects, especially when used long term or at high doses. Side effects include weight gain, increased risk of infection, bone thinning (osteoporosis), and mood changes. Because of these potential side effects, doctors often try to minimize the dose and duration of corticosteroid treatment.

5

What does it mean for someone with polyarteritis nodosa (PAN) to experience a relapse, and why is it significant?

Relapse in polyarteritis nodosa (PAN) refers to the recurrence of disease symptoms after a period of remission or improvement. It's significant because it indicates that the initial treatment was not sufficient to maintain long-term control of the disease. Relapses can manifest as weight loss, ankle swelling, testicular pain, and new-onset hypertension. Managing relapses often requires alternative treatment strategies, such as adalimumab, to regain control of the disease and prevent further complications.

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