ANCA-Negative Granulomatosis of Polyngiitis of Paranasal Sinuses with Cerebellar Involvement

Area of Science:

• Immunology
• Rheumatology
• Neurology

Background:

• Granulomatosis with polyangiitis (GPA) is a rare autoimmune disease affecting small- and medium-sized blood vessels.
• It commonly involves the respiratory tract and kidneys, often associated with anti-neutrophil cytoplasmic antibodies (ANCA).
• However, ANCA positivity is not a prerequisite for diagnosis.

Purpose of the Study:

• To report a rare case of ANCA-negative granulomatosis with polyangiitis.
• To illustrate the diverse clinical manifestations of GPA, including neurological involvement.
• To emphasize the importance of considering GPA even in the absence of ANCA.

Main Methods:

• Case report of a patient diagnosed with granulomatosis with polyangiitis.
• Clinical presentation review focusing on respiratory and neurological symptoms.
• Diagnostic workup including serological tests (ANCA) and imaging.

Main Results:

• The patient presented with sinusitis and was found to have granulomatosis with polyangiitis.
• Crucially, the patient tested negative for anti-neutrophil cytoplasmic antibodies (ANCA).
• Cerebellar involvement was identified, demonstrating atypical disease presentation.

Conclusions:

• This case underscores that granulomatosis with polyangiitis can manifest without ANCA.
• It highlights the potential for GPA to affect the central nervous system, specifically the cerebellum.
• Accurate diagnosis of GPA requires a comprehensive evaluation beyond ANCA status, especially with unusual symptoms.