A Unusual Presentation of Wegener’s Granulomatosis Revealing Perinuclear Antineutrophil Cytoplasmic Antibody Positivity

Wegener’s Granulomatosis (WG), also known as granulomatosis with polyangiitis, is a complex vasculitic condition with diverse systemic manifestations. Often characterized by pauci-immune vasculitis affecting small to medium-sized vessels, WG’s clinical spectrum can be broad and challenging to diagnose. In this case report, we delve into a unique presentation of WG in a 72-year-old female, shedding light on the complexities and diagnostic nuances encountered in her clinical journey.

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Case Presentation

A 72-year-old woman with a history of well-managed type 2 diabetes mellitus and hypertension presented to the hospital with recurrent nasal congestion, headaches, and intermittent facial pain over a two-month period. Initially diagnosed with pansinusitis, her condition took an unexpected turn with the development of bilateral lower limb numbness and weakness. Serial investigations eventually led to a WG diagnosis, highlighting the importance of considering vasculitic conditions even when autoantibody tests yield inconclusive results.

Clinical Manifestations and Diagnostic Challenges

WG’s granulomatous inflammation can manifest across multiple organ systems, with neurological involvement occurring in a significant percentage of cases. The presence of antineutrophil cytoplasmic antibodies (ANCA), specifically perinuclear (p)-ANCA positivity, remains a rare finding. The case emphasizes the need for a comprehensive diagnostic approach, considering the diverse clinical presentations that may not align with typical autoantibody patterns.

Investigation		Initial Value	2 Weeks After	Reference Range
Full blood count	White cell count (× 103/µL)	15.3	10.66	4–11
	Neutrophils (× 103/µL)	12.09	7.79	1.5–7.5
	Hemoglobin (g/dL)	10.7	7.9	12–14
	Mean corpuscular volume (fL)	–	88	80–100
	Mean corpuscular hemoglobin (pg)	–	28.85	27–32
	Platelets (× 103/µL)	386	184	150–400
Inflammatory markers	CRP (mg/L)	177	5	<5
	Erythrocyte sedimentation rate (ESR) (mm in the first hour)	–	110	<20
Renal profile	Serum creatinine (µmol/L)	94	366	53–97.2
	Serum sodium (mmol/L)	129	–	135–145
Urine full report	Pus cells (per high-power field)	3–5	2–4
	Red cells (per high-power field)	10–12	Moderately field full
	Red cell casts	Negative	–
Imaging studies	Ultrasound scan of the abdomen	Grade 1 fatty liver	Normal renal morphology

Discussion: Understanding Wegener’s Granulomatosis

Formerly known as Wegener’s granulomatosis, GPA is an ANCA-associated small vessel vasculitis characterized by necrosis, granulomatous inflammation, and vasculitis. C-ANCA, directed against proteinase 3, is a hallmark, but its absence does not rule out the diagnosis. The disease predominantly affects the upper and lower respiratory tracts and kidneys, leading to a varied clinical presentation, including ocular, gastrointestinal, cardiac, skin, and nervous system manifestations.

Diagnostic Dilemmas and Multidisciplinary Management

The case presented challenges in diagnosis, with inconclusive initial investigations. GPA’s classification criteria and disease states were explored, and the patient’s severe and active disease state guided the choice of treatment. A multidisciplinary approach involving nephrology, pathology, and neurology was crucial for effective management.

Treatment Strategies: Striving for Remission

Early initiation of remission induction is paramount in preventing organ damage. Corticosteroids are foundational, with rituximab or cyclophosphamide recommended for severe cases. Rituximab, preferred for its lower toxicity, demonstrates efficacy in achieving remission. Nonsevere GPA is managed with methotrexate for remission induction, while remission maintenance involves rituximab, methotrexate, azathioprine, or mycophenolate mofetil.

Conclusion: Navigating Wegener’s Granulomatosis

Timely identification and comprehensive treatment are imperative in managing Wegener’s granulomatosis. Despite diagnostic challenges, this case underscores the significance of a multidisciplinary approach and the effectiveness of rituximab in achieving remission. Understanding the varied clinical presentations and treatment nuances is crucial for optimizing outcomes in Wegener’s granulomatosis.

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