Case Presentation:

A 59‐year‐old man with no medical history was referred for admission from a rheumatology clinic where he presented with night sweats, nonproductive cough, exertional dyspnea, nausea, decreased appetite, and loose stools. He also noted raised nodules on his fingers and elbows bilaterally. He was in his usual state of health until 10 months prior to admission when he developed myalgias, arthralgias, fatigue, and unintentional weight loss. He had seen several doctors as an outpatient, but workup was limited. Physical exam was normal. In addition to anemia (hemoglobin of 10.1 g/dL) and acute kidney insufficiency (creatinine of 1.86 mg/dL), pertinent laboratory studies included an increased ESR (117 mm/hour), rheumatoid factor (236 IU/mL), c‐ANCA titer, and anti–proteinase 3 titer. Pertinent negative titers included ANA, p‐ANCA, and antimyeloperoxidase. CXR showed left‐sided peripheral opacities. Chest CT showed multiple peripherally located cavitary masses with diffuse ground glass opacities bilaterally. Granulomatosis with polyangiitis (GPA; formerly known as Wegener's) was suspected, and a sinus CT revealed moderate pansinonasal disease, most severe in the left maxillary sinus. Diagnosis was confirmed after sinus biopsy and drainage, and the patient was empirically started on 30 mg of prednisone. After the biopsy, he exhibited altered mental status and lethargy, arousable only by painful stimuli. Head CT revealed pneumocephalus and acute left frontal cerebral bleeding. Emergent neurosurgical intervention with a craniotomy was performed for decompression. Transnasal biopsy cultures grew fungi morphologically consistent with aspergillus. The patient was treated with amphotericin B and later switched to voriconazole due to concerns over nephrotoxicity. The patient slowly improved, although he had a residual expressive aphasia on discharge to a rehabilitation facility. On follow‐up 2 months later, he was off antibiotics, had persistent aphasia, and was being maintained on prednisone for GPA.


Diagnosis of GPA is based on serologic confirmation of clinical findings that reflect granulomatous involvement of the upper/lower respiratory tracts and kidneys. In our patient, elevated c‐ANCA and anti–proteinase 3 confirmed the diagnosis of GPA that was suggested by imaging studies. However, the co‐occurrence of invasive aspergillosis in the sinuses with seeding into the brain is uncommon. An invasive pulmonary aspergilloma can mimic radiological findings of GPA, thus it is important to exclude fungal and mycobacterial infections prior to immunosuppressive therapy, as they can mimic findings of and coexist with GPA, as in this case.


A strong likelihood of the diagnosis of GPA should not dissuade a clinician from excluding coexisting infections that can mimic granulomatous disease. This case highlights the importance of their rare but important co‐occurrence because greater harm can be caused in this setting when starting a patient on immunosuppressive therapy.