The patient was referred with a working diagnosis of idiopathic orbital inflammatory syndrome (IOIS). The differential diagnosis was extensive, including: infectious (e.g., viral, bacterial, mycobacterial and fungal); inflammatory (e.g., sarcoid, IOIS, ruptured dermoid cyst); collagen vascular disease; and benign or malignant tumors. Excisional biopsy was performed. A 20 mm x 18 mm x 6 mm circumscribed firm mass, with a smooth, pink surface, surrounded by fibrosis, was excised. Histopathologic examination revealed a chronically inflamed, scarred and atrophic lacrimal gland with varying degrees of atrophy (See Figure 2). Some acini were well-preserved with others largely replaced by a diffuse infiltrate of well-differentiated lymphocytes and numerous plasma cells. Occasional sections revealed scarce eosinophils. Flow cytometry demonstrated polytypic B cells and CD5 positive T cells with no immunophenotypic evidence of a lymphoproliferative disorder. The patient was diagnosed with chronic inflammation with fibrosis of the right lacrimal gland. Following excisional biopsy, the patient reported resolution of symptoms and did not require systemic treatment.

Figure 2. Microscopic examination of excisional biopsy. Note a chronically inflamed, scarred and atrophic lacrimal gland. Some acini are well-preserved with others largely replaced by a diffuse infiltrate of well-differentiated lymphocytes and numerous plasma cells. Occasional sections revealed scarce eosinophils.  Figure 3. Renal biopsy. Biopsy revealed nine of 14 glomeruli with active lesions. Six glomeruli show cellular crescent with fibrinoid necrosis. There was no immune complex deposition or anti-glomeruli basement membrane staining by immunofluorescence.

Nine months following excisional biopsy, the patient reported recurrence of RUL swelling associated with headache, vomiting, abdominal pain, abdominal rash, lethargy, dizziness, dyspnea  and coughing. The patient presented to her pediatrician who noted a right-sided flank mass with an overlying rash. Subsequent MRI of the orbits revealed a superolateral orbital lesion increased in size versus initial MRI despite interval excision. Computed tomography imaging of the abdomen revealed a right-sided renal mass. Renal biopsy revealed nine of 14 glomeruli with active lesions (See Figure 3). Six glomeruli showed cellular crescent with fibrinoid necrosis. There was no immune complex deposition or anti-glomeruli basement membrane staining by immunofluorescence.

Based on these developments, the differential diagnosis was narrowed to infectious (e.g., tuberculosis) and inflammatory (e.g., Churg-Strauss syndrome, Wegener’s granulomatosis, microscopic polyangiitis, sarcoidosis, and polyarteritis nodosa). Repeat laboratory analysis revealed hemoglobin of 10.8 g/dL, platelets of 456 x 103/μL, ESR of 70 mm/hr, CRP of 2.92 mg/L and complete metabolic panel within normal limits. Additional studies included urinalysis, revealing trace blood, 4+ protein, and trace leukocytes. Testing for anti-nuclear antibody, cytoplasmic-anti-neutrophil cytoplasmic antibody (c-ANCA) and perinuclear-ANCA (p-ANCA), anti-proteinase 3 (anti-PR3), serum angiotensin converting enzyme, tuberculin skin test and group A strep were negative. Anti-myeloperoxidase (anti-MPO) antibody was positive. Based on these results, the patient was diagnosed with Churg-Strauss syndrome. The patient was subsequently treated with cyclophosphamide and prednisone with improvement in her symptoms.