GRANULOMATOSIS WITH POLYANGIITIS (WEGENER’S GRANULOMATOSIS): A RARE ENTITY PRESENTING WITH UNUSUAL ENDOSCOPIC APPEARANCE

Danrell Yumul, Rafael Chan, Michelle Diwa, Mae Quizon, Geraldine Zamora

Significance: To the authors’ knowledge, after a thorough literature search, this is the first recorded case of granulomatosis with polyangiitis, who presented with gastrointestinal manifestations reported in the Philippines. This case also highlights the importance of early diagnosis with endoscopic biopsy which can lead to early institution of the appropriate treatment that can prevent the irreversible destructive lesions of the disease.

Clinical Presentation: A 37-year-old male, with good baseline capacity and with no known comorbidities was admitted because of a of progressive dysphagia. Pertinent past medical history include a mild COVID 19 infection presenting as sinusitis, otitis media of the left ear requiring myringotomy and recurrent epistaxis since childhood. On further probing, his family history revealed that his paternal uncle was diagnosed to have granulomatosis with polyangiitis presenting as renal failure. Our patient initially presented with migratory joint pains accompanied by sudden appearance of non-blanching purpuric lesions over the lower extremities. In the interim, patient now developed cough with hemoptysis associated with fever, night sweats and progressive dysphagia prompting consultation. On physical exam, the patient had stable vital signs, non-blanching purpura over the lower extremities, multiple brown macules on the trunk, bilateral conjunctival hyperemia, mouth sores on the roof of the mouth, limited range of motion and minimal joint swelling with 70% decreased gross hearing on the left ear, the rest of the physical exam was unremarkable.

Management: Initial work up with chest X-ray revealed hazy densities on the right upper lung field. Computerized tomography scan (CT scan) of the chest showed bilateral pulmonary ground glass opacities with a consideration of infection, pulmonary hemorrhage or cryptogenic organizing pneumonia. At this point, an initial impression of PTB versus GPA was considered. Further diagnostic tests were requested to further narrow down the diagnosis: sputum GeneXpert=negative, anti-dsDNA=negative, RF=normal, ANA=negative, anti-MPO=negative, anti-PR3=positive, P-ANCA=negative, c-ANCA=positive which were consistent with granulomatosis with polyangiitis. Due to persistence of dysphagia, patient underwent Endoscopic Gastroduodenoscopy (EGD), which revealed a circumferential denuded esophageal mucosa with polypoid lesions from the mid to the distal portion of the esophagus (Figure 1) and erosive gastritis. Multiple biopsies taken which revealed vasculitis with granulomatous process consistent with GPA with gastrointestinal involvement (Figure 2). Dermoscopy was done and showed globular red dots in orange-brown background consistent with vasculitis. Skin punch biopsy was also done, which revealed an immunofluorescence report of fibrinogen positive (++) perivascular deposition, C3 positive (++) perivascular deposition, and negative immunoglobulin G (IgG), immunoglobulin M (IgM), and immunoglobulin A (IgA) which were consistent with vasculitis. The patient was then given three cycles of Methylprednisolone pulse therapy (MPPT) followed by Rituximab infusion once a week for four weeks. Methotrexate was also given after completion of Rituximab infusion. The patient had significant improvement of his symptoms after the treatment and there were no other remarkable findings during his outpatient visits.

Conclusion: Although GPA predominantly affects respiratory tract and renal systems, some patients have cutaneous and gastrointestinal involvement which could serve as a potentially more accessible sites to obtain tissue biopsy to confirm the diagnosis leading to prompt treatment and preventing life-threatening consequences of this systemic disease.

Lorem ipsum dolor sit amet, consectetur adipiscing elit. Etiam turpis eros, lacinia eget elementum commodo, laoreet a nisi. Mauris porttitor lectus sed dolor lacinia viverra. Donec lobortis posuere purus, vitae volutpat orci fringilla vitae. Quisque a quam non nunc convallis cursus.

All the manuscripts must be submitted on line thru JRRE Secretariat (jrre2016@yahoo.com.ph). There are no article processing charges for submission, editing, review and publication.

View Instructions