fetching data ... 
Background: Steroid free remission in organ threatening ANCA associated vasculitis is currently described only with Avacopan, a C5a receptor inhibitor. However, this drug is not yet available for use in the third world.(1)
Objectives: To describe a clinical case of granulomatous polyangiitis with orbital pseudotumor right eye with optic atrophy, who developed steroid induced central serous retinopathy in the other eye, necessitating a steroid free treatment regime.
Methods: A 45year old man was admitted to the department of clinical immunology, JIPMER in January 2021 with Orbital pseudotumor Rt eye since 2019 causing optic atrophy, treated with high dose steroids for more than a year. He developed steroid induced central serous retinopathy of left eye, necessitating withdrawal of steroids, leading to disease flare in the form of painful right orbital inflammation, acute glomerulonephritis, vasculitic ulcers over leg and lung nodules. His PR3 ANCA titers were elevated at the time of disease flare. His sputum infectious workup was negative for bacteria/fungus/tuberculosis. He was treated with 10 cycles of radiotherapy to right eye for debulking followed by exenteration. As he had pseudomonal infection in the post operative orbital cavity at the time of flare, 2 monthly doses of IVIG 2g/kg was given; infection treated with antibiotics and regular dressing. After resolution of infection 6 doses of cyclophosphamide as per EUVAS protocol and Rituximab 2 gm was given along with the first dose of Cyclophosphamide.
Results: At 48 weeks of follow up, he continues to be in sustained clinical remission without steroids. He has received additional 500 mg of rituximab at 28 weeks. His lung nodules and vasculitic ulcers of leg healed over 34 weeks. He has developed saddle nose deformity for which reconstruction is planned.
Conclusion: Steroid free remission in GPA may be possible without Avacopan.
REFERENCES:
[1]Jayne DRW, Merkel PA, Schall TJ, Bekker P. Avacopan for the Treatment of ANCA-Associated Vasculitis. N Engl J Med. 2021 Feb 18;384(7):599–609.
Investigations:
| Week 0 | Week 28 | Week 48 | |
|---|---|---|---|
| Hemoglobin(g/dl ) | 5.5 | 7.8 | 10.7 |
| Creatine (mg/dl ) | 1.43 | 1.35 | 1.0 |
| Albumin (mg/dl ) | 1.9 | 2.5 | 3.8 |
| Urine analysis | Protein:2+RBCs:50 -100/hpf | Protein:1+RBCs: 10-15/hpf | Protein: traceRBCs: <10/hpf |
| CECT Thorax | Left upper lobe solitary nodule | Resolved | |
| MRI orbit | RT orbital pseudotumor with optic atrophy | S/P exenteration | |
| Renal Biopsy | Focal proliferative sclerosing glomerulonephritis; No crescents. | ||
| Orbital tissue biopsy | Palisading granuloma | ||
| PR3 ANCA(ELISA ) | 57.63 | ||
| MPO ANCA(ELISA ) | negative | ||
| ANA | Negative | ||
| C3/C4 | 1.61/0.32 (normal) | ||
| ACE levels | 25.3 (normal) | ||
| IgG4 | 2.73 | ||
| BVAS | 20 | 5 | 0 |
| VDI | 3 | 3 | 3 |
Abbreviations: hpf: high power field, CECT: Contrast enhanced computed tomography, PR3: proteinase3, MPO: Myeloperoxidase, ANA: Antinuclear antibodies, ACE: Angiotensin converting enzyme, BVAS: Birmingham vasculitis activity score, VDI: Vasculitis damage index
Clinical features of GPA
Disclosure of Interests: None declared