A Unusual Presentation of Wegenerโ€™s Granulomatosis Revealing Perinuclear Antineutrophil Cytoplasmic Antibody Positivity

A Unusual Presentation of Wegenerโ€™s Granulomatosis Revealing Perinuclear Antineutrophil Cytoplasmic Antibody Positivity

Wegenerโ€™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.

InvestigationInitial Value2 Weeks AfterReference Range
Full blood countWhite cell count (ร— 103/ยตL)15.310.664โ€“11
Neutrophils (ร— 103/ยตL)12.097.791.5โ€“7.5
Hemoglobin (g/dL)10.77.912โ€“14
Mean corpuscular volume (fL)โ€“8880โ€“100
Mean corpuscular hemoglobin (pg)โ€“28.8527โ€“32
Platelets (ร— 103/ยตL)386184150โ€“400
Inflammatory markersCRP (mg/L)1775<5
 Erythrocyte sedimentation rate (ESR) (mm in the first hour)โ€“110<20
Renal profileSerum creatinine (ยตmol/L)9436653โ€“97.2
Serum sodium (mmol/L)129โ€“135โ€“145
Urine full reportPus cells (per high-power field)3โ€“52โ€“4 
Red cells (per high-power field)10โ€“12Moderately field full 
Red cell castsNegativeโ€“ 
Imaging studiesUltrasound scan of the abdomenGrade 1 fatty liverNormal renal morphology

Discussion: Understanding Wegenerโ€™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 Wegenerโ€™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 Wegenerโ€™s granulomatosis.

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