A 58-year-old woman presented with progressive painless visual loss in both eyes for 1 month. She had low grade fever, non-productive cough, and weight loss for 6 months.
Her medical history was notable for right Bell's palsy and bilateral hearing loss 1 year ago and was improved after a short course of oral corticosteroid. She denied any history of tuberculosis contact. She acknowledged no family history of cancer, tuberculosis, nor autoimmune diseases.
Physical examination revealed body temperature 37.7  C, blood pressure 133/80 mmHg, pulse rate 96/minute, and respiratory rate 18/minute. Ophthalmic examination revealed peripheral ulcerative keratitis, necrotizing scleritis, impending corneal perforation on both eyes, right visual acuity (VA) 3/60, left VA 6/24, normal intraocular pressure (IOP). The examination of ear-nose-throat (ENT), cardiovascular system, lung, abdomen, extremities, lymph nodes, skin and neurological system were unremarkable.
The laboratory examination showed hemoglobin 12.2 g/dL, white blood cell count 12,930 cell/mm3 (74.6% neutrophils, 18% lymphocytes, 5.7% monocytes, 0.9% eosinophils), platelet count 927,000/mm3, Cr 1.0 mg/dL, BUN 15.2 mg/dL, globulin 3.7 g/dL, albumin 3.8 g/dL, and no hepatitis. Urinalysis revealed trace protein, WBC 0-1 cell/HPF, and RBC 0-1 cell/HPF. Serum VDRL, TPHA, hepatitis B, hepatitis C, and anti-HIV antibodies were negative. Anti-RF antibody was 124.60 IU/ml (0-20 IU/ml) but Anti-CCP antibody was <7 U/mL. ANA was positive for nucleolar pattern with titer 1:320. The c-ANCA (IFA) and anti-PR3 antibody (western blotting) were positive.
The chest radiograph revealed multiple mass-like lesions. A chest CT showed two heterogenous enhancing masses, one 3.6 cm in size located at the lateral segment of the right middle lobe and the other was 4.4 cm in size located at the superior segment of the right lower lobe. A CT-guided lung mass biopsy was done at right lower lobe lesion. The pathological findings were one focus of necrotizing vasculitis. The AFB stain, gram stain, GMS stain, PAS stain, and cultures were all negative.
One-gram methylprednisolone was given for 3 days as initial therapy, followed by monthly intravenous 800-mg cyclophosphamide and prednisolone for 6 months. During treatment, the ophthalmic and pulmonary lesions were improved. After 6 months of therapy, she developed new mass-like lesions at right lower lungs without ophthalmic lesion. Bronchoscopy was done and revealed an ulcerative lesion along the carina through the right main bronchus. All microbiological testing was negative.
A right lower lobe wedge resection was performed due to isolated pulmonary lesions, and showed epithelioid histiocyte aggregation with central necrosis, eosinophils and multinucleated giant cells, lymphoplasmacytic infiltration in the vascular wall, compatible with granulomatous inflammation with destructive leukocytic angiitis. Special strains for AFB, GMS and PAS revealed no microorganisms (Fig. 1b). The final diagnosis was GPA with diffuse necrotizing scleritis, central airway, and lungs involvements by ACR/EULAR 2017 provisional classification criteria. Azathioprine and oral prednisolone were given for maintenance of remission. She was in remission state.
Chest CT showed the largest pulmonary mass at lateral segment of right middle lobe.