A Case of Granulomatosis with Polyangiitis: Consequences of Delayed Diagnosis in a Life-threatening Malady

Abstract

Granulomatosis with polyangiitis (GPA) is one of three described anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV). Early diagnosis and treatment of GPA is paramount, as it may help prevent irreversible end-organ damage, especially renal and pulmonary failure.A 72-year-old male with a past medical history of lung adenocarcinoma in remission, chronic sinusitis status-post multiple sinus surgeries, and coronary artery disease presented with shortness of breath, dark urine, and asymmetric polyarthralgias. He had an acute kidney injury, leukocytosis, with urinalysis demonstrating pyuria and hematuria, without casts. Chest imaging showed cavitary nodular opacities in addition to interval increase of existing nodules compared to the most recent scan one month prior. His acute kidney injury progressed to renal failure requiring hemodialysis, and he developed an inflammatory polyarthritis. GPA was suspected clinically so he was started on high-dose intravenous corticosteroids, and subsequently plasmapheresis and rituximab. Serology returned with highly positive proteinase-3 antibodies, and cytoplasmic ANCA positivity on immunofluorescence. Renal biopsy demonstrated severely active pauci-immune glomerulonephritis. Several months after discharge, the patient passed away from gram positive bacteremia.This patient’s recurrent sinusitis, pulmonary nodules, and subsequent renal failure were highly suggestive of GPA. A biopsy is recommended to confirm the diagnosis of GPA, but treatment should not be delayed if there is a high index of suspicion for the disease. Induction therapy with corticosteroids combined with rituximab or cyclophosphamide has significantly decreased the mortality of patients with GPA.Patients with GPA often have preceding history of nasopharyngeal and upper airway disease, and can present with fluctuating pulmonary infiltrates. Early recognition and treatment of patients with GPA can prevent life-threatening complications and reduce mortality.

A rare case of coexistence of Wegener’s granulomatosis and pulmonary tuberculosis with subsequent development of thrombosis of the cerebral veins

“…An abnormal CXR, an abnormal urinary sediment and an indicative kidney biopsy were therefore reliable criteria to diagnose GPA in our patient, as recommended by the American College of Rheumatology [ 13 , 14 ].…”

“…According to the American College of Rheumatology two or more of the following criteria are needed to diagnose GPA with a specificity of 92%: nasal/oral inflammation, abnormal urinary sediment, abnormal CXR and suggestive kidney histology [ 13 , 21 ]. In our patient an abnormal CXR, an abnormal urinary sediment and a typical kidney histology were the criteria employed to diagnose GPA [ 13 , 14 ].…”

“…Prednisolone and cyclophosphamide are the gold standard to treat severe forms of GPA. Since GPA can be ANCA negative in 10% cases, immunosuppressive treatment should be started as soon as possible in highly suspicious GPA, irrespective of auto-antobody serology [ 31 ] Immunosuppressive treatment is in fact critical to decrease morbidity and mortality in the long term [ 14 ]. Although the latter treatment regimen allows remission in over 90% of GPA patients, the kidney function should be consistently verified before treatment start [ 13 ].…”

“…An abnormal CXR, an abnormal urinary sediment and an indicative kidney biopsy were therefore reliable criteria to diagnose GPA in our patient, as recommended by the American College of Rheumatology [13,14]. Although no endobronchial lesions could be observed at bronchoscopy and the purified protein derivative (PPD) skin test was negative, broncho-alveolar lavage (BAL) smear turned up positive for acid-fast bacilli, with TB being subsequently confirmed at PCR and (after 40 days) culture.…”