Overlap between systemic sclerosis and polyarteritis nodosa: A case report

Area of Science:

• Rheumatology
• Immunology
• Gastroenterology

Background:

• Systemic sclerosis (SSc) is a connective tissue disease marked by vasculopathy, inflammation, autoimmunity, and fibrosis.
• Frank vasculitis is an uncommon manifestation in SSc patients.

Purpose of the Study:

• To report a rare case of co-existing Systemic Sclerosis and Polyarteritis Nodosa.
• To discuss the diagnostic challenges and treatment modifications required for this rare association.

Main Methods:

• A case presentation of a 36-year-old male with limited cutaneous SSc.
• Diagnostic workup included serological tests, imaging (ultrasound, Doppler, Fibroscan), and assessment for vasculitis and liver involvement.
• Treatment involved high-dose corticosteroids, plasmapheresis, cyclophosphamide, and azathioprine.

Main Results:

• The patient presented with necrotic ulcers, neuropathy, and testicular pain, indicative of vasculitis.
• Liver evaluation revealed fibrosis and ascites, with elevated liver enzymes.
• Diagnosis of polyarteritis nodosa was established, and the patient responded well to the intensive immunosuppressive therapy.

Conclusions:

• The association of Systemic Sclerosis and Polyarteritis Nodosa is exceedingly rare.
• Co-existence necessitates tailored treatment strategies, often involving potent immunosuppression, to manage the severe manifestations of vasculitis in SSc patients.