P29 Use of novel biologic therapies for arthropathy affecting common variable immunodeficiency patients. (20th April 2020)

• Record Type: Journal Article
• Authors: Henneberry, Robert; Burns, Siobhan; Stratton, Richard; Lowe, David M
• Is Part Of: Rheumatology. Volume 59(2020)Supplement 2
• Publication Date: 2020-04-20
• Subjects: Rheumatism -- Periodicals; Rheumatology -- Periodicals; 616.723005
• DOI: 10.1093/rheumatology/keaa111.028
• Languages: English
• ISSNs: 1462-0324

Abstract

Abstract: Background: CVID is the most common adult immunodeficiency with an incidence of 1:25000. It is characterised by hypogammaglobulinaemia and recurrent bacterial infections. Patients also develop granulomatous interstitial lung disease and are at increased risk of lymphoma. Autoimmune disease, most commonly AIHA, ITP and thyroiditis, occurs at a rate of 25%. Previous studies suggested that 3% of patients have inflammatory arthritis, but from our experience a higher proportion complain of chronic arthralgias. Treating arthropathy in CVID is challenging; examination and inflammatory markers may be normal and there is concern about the risk of infections if using methotrexate or biologics. Methods: We present a series of 7 patients with arthropathy in CVID. We chose DMARDs based on the individual case, tailor-made to the patients clinical and immunologic status. Results: Patients had longstanding symptoms, with a median duration of 11 years. All patients had disabling joint pain, in three it was the most prominent symptom of their illness. All patients had pain/swelling in a typical symmetrical small joint polyarthritis distribution. Fatigue was also prominent. On examination three patients had clear evidence of synovitis, one had tenderness but no swelling, and three had no evidence of synovitis; there was no deformity. No patient had positive autoantibodies. Three had chronically raised CRP/ESR, one had raised inflammatory markers during flares, but the other three had normal markers. Prednisolone (10-15 mg) controlled arthritic symptoms. There was little response to conventional DMARDS or rituximab. Two patients treated with abatacept showed improvement in symptoms, but one died from PJP pneumonia. Two patients treated with baricitinib showed improvement in symptoms but one relapsed after 5 months (Table 1). Conclusion: Arthopathy is a common, and probably under-diagnosed, feature of CVID. It is difficult to treat, with a poor response seen to most DMARDs. We have seen promising results with Baricitinib and propose using it first line in patients with disabling rheumatic symptoms. There may be a role for abatacept in co-existent severe joint and lung disease. A striking proportion, 4/7 (versus 10% in general CVID population), of patients had a causal genetic mutation identified. Arthropathy may, therefore, push immunologists towards genetic testing. Disclosures: R. Henneberry None. S. Burns None. R. Stratton None. D.M. Lowe None.