Heba M. Bintalib ^1,2,3* Sofia Grigoriadou ⁴ Smita Patel ⁵ Leman Mutlu ⁶ Kavitha Sooriyakumar ⁷ Prashantha Vaitla ⁸ Elizabeth Mcdermott ⁸ Elizabeth Drewe ⁸ Cathal Steele ⁹ Manisha Ahuja ¹⁰ Tomaz Garcez ¹¹ Mark Gompels ¹² Alexandros Grammatikos ¹² Archana Herwadkar ¹³ Rehana Ayub ¹⁴ Neil Halliday ^15,16 Siobhan O. Burns ^17,18 John R. Hurst ¹⁹ Sarah Goddard ²⁰

• ¹ University College London, London, United Kingdom
• ² Department of Respiratory Care, College of Applied Medical Sciences-Jeddah, King Saud bin Abdulaziz University for Health Sciences, Jeddah, Saudi Arabia
• ³ King Abdullah International Medical Research Centre, Jeddah, Saudi Arabia
• ⁴ Department of Immunology, Royal London Hospital, Barts Health NHS Trust, London, England, United Kingdom
• ⁵ Oxford University Hospitals NHS Trust, Oxford, England, United Kingdom
• ⁶ East Kent Hospitals University Nhs Foundation Trust, Ashford, United Kingdom
• ⁷ Department of Immunology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, United Kingdom
• ⁸ Clinical Immunology and Allergy Department, Nottingham University Hospitals NHS Trust, Nottingham, England, United Kingdom
• ⁹ Regional Immunology Service of Northern Ireland, Belfast Health and Social Care Trust, Belfast, United Kingdom
• ¹⁰ Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, United Kingdom
• ¹¹ Manchester University NHS Foundation Trust (MFT), Manchester, United Kingdom
• ¹² The Bristol NHS Immunology Allergy Centre, Southmead Hospital, Bristol, United Kingdom
• ¹³ Immunology Department, Division of Surgery & Tertiary Medicine, Salford Royal NHS Foundation Trust, Salford, England, United Kingdom
• ¹⁴ Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom
• ¹⁵ Institute for Liver and Digestive Health, University College London, London, England, United Kingdom
• ¹⁶ Sheila Sherlock Liver Centre, Royal Free London NHS Foundation Trust, London, United Kingdom
• ¹⁷ Department of Immunology, Royal Free London NHS Foundation Trust, London, United Kingdom
• ¹⁸ Institute of Immunity and Transplantation, University College London, London, England, United Kingdom
• ¹⁹ UCL Respiratory, University College London,, London, United Kingdom
• ²⁰ Department of Immunology, University Hospitals North Midlands, University Hospitals of North Midlands NHS Trust, Stoke-on-Trent, Stoke-on-Trent, United Kingdom

Background: Common Variable Immunodeficiency Disorders (CVID) encompass a spectrum of immunodeficiency characterised by recurrent infections and diverse non-infectious complications (NICs). This study aimed to describe the clinical features and variation in NICs in CVID with and without interstitial lung disease (ILD) from a large UK national registry population.Methods: Retrospective, cross-sectional data from a UK multicentre database (previously known as UKPIN), categorising patients into those with CVID-ILD and those with NICs related to CVID but without pulmonary involvement (CVID-EP; EP= extra-pulmonary involvement only).Results: 129 patients were included. Chronic lung diseases, especially CVID-ILD, are prominent complications in complex CVID, occurring in 62% of the cohort. Bronchiectasis was common (64% of the cohort) and associated with greater pulmonary function impairment in patients with CVID-ILD compared to those without bronchiectasis. Lymphadenopathy and the absence of gastrointestinal diseases were significant predictors of ILD in complex CVID. Although the presence of liver disease did not differ significantly between the groups, nearly half of the CVID-ILD patients were found to have liver disease. Patients with CVID-ILD were more likely to receive immunosuppressive treatments such as rituximab and mycophenolate mofetil than the CVID-EP group, indicating greater need for treatment and risk of complications.This study highlights the significant burden of CVID-ILD within the CVID population with NICs only. The lungs emerged as the most frequently affected organ, with ILD and bronchiectasis both highly prevalent. These findings emphasise the necessity of a comprehensive and multidisciplinary approach in managing CVID patients, considering their susceptibility to various comorbidities and complications.