Female With Hypertensive Emergency Later Found to Have ANCA-Associated Vasculitis: A Case Report

Area of Science:

• Nephrology
• Rheumatology
• Internal Medicine

Background:

• Granulomatosis with polyangiitis (GPA) is a necrotizing vasculitis of small vessels, often affecting respiratory tracts and kidneys.
• Typical GPA symptoms include sinusitis, respiratory issues, and glomerulonephritis, with positive ANCA tests in most cases.
• Treatment focuses on remission induction and maintenance therapy.

Observation:

• A 48-year-old female presented with a hypertensive emergency (BP > 220 mmHg), severe headache, and cough, initially treated as pneumonia.
• Despite antihypertensives, her blood pressure remained high, accompanied by hematuria and anemia, suggesting underlying vasculitis.
• Clinical history of joint pain, sinus infections, and rash, along with positive PR3 antibodies and c-ANCA, raised suspicion for GPA.

Findings:

• Kidney biopsy confirmed acute necrotizing pauci-immune glomerulonephritis, diagnostic of GPA.
• Steroids and rituximab therapy led to significant clinical improvement and blood pressure control.
• Pulmonary findings mimicked infection, and cutaneous findings correlated with joint and renal involvement.

Implications:

• This case highlights a rare GPA presentation as hypertensive emergency, emphasizing the need for awareness in atypical cases.
• Early diagnosis and aggressive immunosuppression are critical to prevent irreversible renal damage in GPA.
• The complex interplay of pulmonary, renal, and cutaneous manifestations underscores GPA's varied clinical spectrum.