TB or Not TB: A Case Report of Granulomatosis with Polyangiitis
Location
Philadelphia, PA
Start Date
11-5-2022 1:00 PM
End Date
11-5-2022 4:00 PM
Description
Introduction:
Granulomatosis with Polyangiitis (GPA, formerly known as Wegener’s granulomatosis) is an antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis whose diagnosis often hinges on clinician identification of multisystemic disease. In the classic triad, patients with GPA present with upper airway involvement, lower respiratory tract involvement, and glomerulonephritis. In the absence of renal pathology, differentiation of GPA from tuberculosis can pose a challenge.
Case Presentation:
Here we present the case of a 23-year-old woman who presented with complaints of 2 weeks of right eye redness and double vision, 1 month of dyspnea on exertion with nasal congestion and non-productive cough, 1 year of hearing loss, significant weight loss, and arthralgias. She had no significant past medical history and immigrated to America from Honduras 5 to 7 months prior to presentation. Vitals on arrival were unremarkable. Labs were significant for WBC 10.6 with 6% bands, Hgb 10.2, platelets 788, CRP 9.4, and ESR 108 with rouleaux present. Pertinent physical exam findings include right eye proptosis with left eyelid ptosis, weakness of left upward and medial gaze with mild anisocoria, tongue deviation to right, bilateral acute otitis media, restricted range of motion of left upper extremity with 3/5 strength, and lungs clear to auscultation bilaterally. MRI revealed basilar meningitis and on chest x-ray, numerous cavitary lung lesions were identified. CT chest showed multifocal bilateral masslike opacities, some of which were cavitated, with mediastinal adenopathy and the patient was suspected to have tuberculosis. She was placed on zosyn, rifampin, and isoniazid while lab results were pending. In the interim, she underwent a lumbar puncture, which was remarkable only for elevated glucose in CSF of 73, leading us to reconsider the likelihood of a tuberculosis diagnosis. Blood cultures were negative throughout the duration of stay, as were RPR, HIV, and Lyme titers. Resulting labs were positive for rheumatoid factor, ANA, c-ANCA, anti-PR-3, and a polyclonal increase in gamma globulins, with a negative tuberculosis QuantiFERON test, at which time GPA became the leading differential. She was recommended for lung biopsy via thoracoscopic approach with thoracic surgery, which confirmed diagnosis of alveolar hemorrhage and capillaritis variant of GPA. She was started on oral steroids as well as topical prednisone and timolol eye drops. She has been recommended to begin rituximab for long term management.
Conclusion:
When managing pulmonary cavitary lesions and symptoms of meningitis, clinicians must maintain a wide differential. Both TB and GPA should be kept in the differential for patients showing signs of vasculitis with high risk of TB exposure, as TB and GPA can co-exist. Quantiferon Gold and c-ANCA are simple laboratory studies for differentiating the two conditions, but can take several days to obtain results. In cases of limited-GPA in which renal function is intact, patients are still at high risk for development of renal failure, making swift treatment with steroids paramount in importance.
TB or Not TB: A Case Report of Granulomatosis with Polyangiitis
Philadelphia, PA
Introduction:
Granulomatosis with Polyangiitis (GPA, formerly known as Wegener’s granulomatosis) is an antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis whose diagnosis often hinges on clinician identification of multisystemic disease. In the classic triad, patients with GPA present with upper airway involvement, lower respiratory tract involvement, and glomerulonephritis. In the absence of renal pathology, differentiation of GPA from tuberculosis can pose a challenge.
Case Presentation:
Here we present the case of a 23-year-old woman who presented with complaints of 2 weeks of right eye redness and double vision, 1 month of dyspnea on exertion with nasal congestion and non-productive cough, 1 year of hearing loss, significant weight loss, and arthralgias. She had no significant past medical history and immigrated to America from Honduras 5 to 7 months prior to presentation. Vitals on arrival were unremarkable. Labs were significant for WBC 10.6 with 6% bands, Hgb 10.2, platelets 788, CRP 9.4, and ESR 108 with rouleaux present. Pertinent physical exam findings include right eye proptosis with left eyelid ptosis, weakness of left upward and medial gaze with mild anisocoria, tongue deviation to right, bilateral acute otitis media, restricted range of motion of left upper extremity with 3/5 strength, and lungs clear to auscultation bilaterally. MRI revealed basilar meningitis and on chest x-ray, numerous cavitary lung lesions were identified. CT chest showed multifocal bilateral masslike opacities, some of which were cavitated, with mediastinal adenopathy and the patient was suspected to have tuberculosis. She was placed on zosyn, rifampin, and isoniazid while lab results were pending. In the interim, she underwent a lumbar puncture, which was remarkable only for elevated glucose in CSF of 73, leading us to reconsider the likelihood of a tuberculosis diagnosis. Blood cultures were negative throughout the duration of stay, as were RPR, HIV, and Lyme titers. Resulting labs were positive for rheumatoid factor, ANA, c-ANCA, anti-PR-3, and a polyclonal increase in gamma globulins, with a negative tuberculosis QuantiFERON test, at which time GPA became the leading differential. She was recommended for lung biopsy via thoracoscopic approach with thoracic surgery, which confirmed diagnosis of alveolar hemorrhage and capillaritis variant of GPA. She was started on oral steroids as well as topical prednisone and timolol eye drops. She has been recommended to begin rituximab for long term management.
Conclusion:
When managing pulmonary cavitary lesions and symptoms of meningitis, clinicians must maintain a wide differential. Both TB and GPA should be kept in the differential for patients showing signs of vasculitis with high risk of TB exposure, as TB and GPA can co-exist. Quantiferon Gold and c-ANCA are simple laboratory studies for differentiating the two conditions, but can take several days to obtain results. In cases of limited-GPA in which renal function is intact, patients are still at high risk for development of renal failure, making swift treatment with steroids paramount in importance.