Presentation
Decline in overall health, along with feelings of tiredness and exhaustion. Additionally, the patient is experiencing headaches as well as binocular diplopia.
Patient Data
A Portal venous CT scan of the chest and abdomen revealed:
multiple necrotic lung nodules, the largest in the left lower lobe
There is smooth pachymeningeal thickening (observed in DWI, FLAIR C+, and 3DT1 C+) and enhancement of the right supratentorial convexity, which also involves the right tentorium cerebelli and cavernous sinus.
The rhinopharynx appears to be hypertrophied, and there is effusion in the mastoid cells, which is likely related to Eustachian tube dysfunction.
The left maxillary sinus shows thickened walls and mucosal thickening, which are consistent with chronic sinusitis.
Enhancement along the right foramen ovale (V3 mandibular branch of trigeminal nerve).
After imaging, possible lung cancer with dural metastases and granulomatous disease were considered.
Meanwhile, the patient developed kidney failure.
Further investigations were conducted, including:
EBUS with 10L node biopsy. The initial histopathology report suggested the presence of a metastatic node from non-small cell lung cancer. However, after considering all clinical, biological, and radiological data, the second analysis could not confirm this report.
Urinalysis. Results showed proteinuria and microscopic hematuria.
Renal biopsy, which revealed proliferative glomerulonephritis with foci of necrosis and necrotizing vasculitis. The presence of associated granuloma was doubtful.
c-ANCA (PR3) positivity
After being diagnosed with granulomatosis with polyangiitis (formerly known as Wegener's disease), the patient received immunosuppressive therapy. He was discharged and returned a few months later for clinical and imaging follow-up, including brain MRI and chest CT.
After six months of treatment, a non-contrast chest CT scan revealed that the lung nodules and adenopathy had almost completely disappeared, except for a small pseudonodule in the left lower lobe.
Following six months of treatment, a follow-up brain MRI showed almost complete regression of pachymeningeal thickening and enhancement with only slight residual pachymeningeal thickening of the right convexity. Additionally, there was slight thickening and contrast enhancement on the right tentorium cerebelli, which appeared hypointense on T2-WI. The rhinopharynx currently displays a normal appearance.
Case Discussion
Granulomatosis with polyangiitis (GPA) affects small to medium-sized arteries, capillaries, and veins, especially in the respiratory system and kidneys.
Symptoms vary depending on which part of the body is affected, but common symptoms include cough, shortness of breath, sinus pain, and kidney problems. Diagnosis is based on a combination of physical examination, laboratory tests, imaging studies, and biopsy. Radiology is essential to determine the extent of the disease and monitor its progression. Treatment usually involves a combination of immunosuppressive medications and steroids. With timely and appropriate treatment, the 5-year survival rate for GPA is around 70-80%.
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