From the Guidelines
Polyarteritis Nodosa (PAN) is a rare and serious autoimmune disorder characterized by inflammation of the blood vessels, which can lead to organ damage and failure if left untreated 1.
Key Characteristics
The condition typically affects medium-sized arteries, leading to a range of symptoms including:
- Abdominal pain
- High blood pressure
- Kidney damage
- Nerve damage
- Skin lesions
Diagnosis
Diagnosis of PAN is often based on a combination of clinical signs, laboratory tests, and imaging studies, including:
- Abdominal vascular imaging to aid in establishing a diagnosis and determining the extent of disease 1
- Deep-skin biopsy specimen to aid in establishing a diagnosis in patients with suspected PAN involving the skin 1
- Combined nerve and muscle biopsy to aid in establishing a diagnosis in patients with suspected PAN and peripheral neuropathy 1
Treatment
The management of PAN typically involves a combination of immunosuppressive medications, including:
- Glucocorticoids (GCs) such as prednisolone to reduce inflammation and suppress the immune system 1
- Cyclophosphamide to induce remission in patients with severe PAN 1
- Other non-GC immunosuppressive agents such as methotrexate or azathioprine to maintain remission and prevent relapse 1
A possible treatment regimen could include:
- IV pulse GCs for patients with newly diagnosed active, severe PAN 1
- Cyclophosphamide and high-dose GCs for patients with newly diagnosed active, severe PAN 1
- Non-GC immunosuppressive agents and GCs for patients with newly diagnosed active, nonsevere PAN 1
It is essential to monitor the patient's response to treatment and adjust the regimen as needed. Additionally, considering the complexity of the condition, consultation with a rheumatology specialist is highly recommended for personalized care and management.
From the Research
Definition and Diagnosis of Polyarteritis Nodosa
- Polyarteritis nodosa is a systemic disease characterized by necrotizing vasculitis in medium- and small-sized vessels, as described in a case study published in 2.
- The diagnosis of polyarteritis nodosa should be considered in patients with chronic polyarthritis associated with cutaneous vasculitis, particularly in cases triggered by streptococcal infection 2.
- Skin biopsy can reveal necrotizing vasculitis, which is compatible with polyarteritis nodosa, as seen in the case of a 4-year-old girl with chronic polyarthritis and cutaneous lesions 2.
Clinical Presentation
- Polyarteritis nodosa can present with chronic polyarthritis, morning stiffness, and cutaneous lesions, such as painful subcutaneous nodules and erythematous-violaceous lesions 2.
- The disease can also cause systemic symptoms, including high fever, malaise, myalgia, anorexia, and weight loss 2.
- In some cases, polyarteritis nodosa can lead to severe functional disability and proteinuria, as seen in the case study published in 2.
Differential Diagnosis
- Polyarteritis nodosa should be differentiated from other diseases that present with polyarthritis, such as rheumatoid arthritis, infectious diseases, and granulomatous mastitis 3, 4, 5.
- A thorough patient history, clinical presentation, and diagnostic investigations are necessary to establish an accurate diagnosis and differentiate polyarteritis nodosa from other conditions 5.