Sarcoidosis versus Granulomatous and Lymphocytic Interstitial Lung Disease in Common Variable Immunodeficiency: A Comparative Review
Helena Buso,
Claudia Discardi,
Patrick Bez,
Francesco Muscianisi,
Jessica Ceccato,
Cinzia Milito,
Davide Firinu,
Nicholas Landini,
Mark G. Jones,
Carla Felice,
Marcello Rattazzi,
Riccardo Scarpa,
Francesco Cinetto
Affiliations
Helena Buso
Rare Diseases Referral Center, Internal Medicine 1, Department of Medicine (DIMED), AULSS2 Marca Trevigiana, Ca’ Foncello Hospital, University of Padova, 35124 Padova, Italy
Claudia Discardi
Rare Diseases Referral Center, Internal Medicine 1, Department of Medicine (DIMED), AULSS2 Marca Trevigiana, Ca’ Foncello Hospital, University of Padova, 35124 Padova, Italy
Patrick Bez
Rare Diseases Referral Center, Internal Medicine 1, Department of Medicine (DIMED), AULSS2 Marca Trevigiana, Ca’ Foncello Hospital, University of Padova, 35124 Padova, Italy
Francesco Muscianisi
Rare Diseases Referral Center, Internal Medicine 1, Department of Medicine (DIMED), AULSS2 Marca Trevigiana, Ca’ Foncello Hospital, University of Padova, 35124 Padova, Italy
Jessica Ceccato
Haematology and Clinical Immunology Unit, Department of Medicine (DIMED), University of Padova, 35124 Padova, Italy
Cinzia Milito
Department of Molecular Medicine, “Sapienza” University of Rome, 00161 Rome, Italy
Davide Firinu
Department of Medical Sciences and Public Health, University of Cagliari, 09124 Cagliari, Italy
Nicholas Landini
Department of Radiological, Oncological and Pathological Sciences, Policlinico Umberto I Hospital, “Sapienza” University of Rome, 00161 Rome, Italy
Mark G. Jones
Clinical and Experimental Sciences, Faculty of Medicine, University of Southampton, Southampton SO16 YD, UK
Carla Felice
Rare Diseases Referral Center, Internal Medicine 1, Department of Medicine (DIMED), AULSS2 Marca Trevigiana, Ca’ Foncello Hospital, University of Padova, 35124 Padova, Italy
Marcello Rattazzi
Rare Diseases Referral Center, Internal Medicine 1, Department of Medicine (DIMED), AULSS2 Marca Trevigiana, Ca’ Foncello Hospital, University of Padova, 35124 Padova, Italy
Riccardo Scarpa
Rare Diseases Referral Center, Internal Medicine 1, Department of Medicine (DIMED), AULSS2 Marca Trevigiana, Ca’ Foncello Hospital, University of Padova, 35124 Padova, Italy
Francesco Cinetto
Rare Diseases Referral Center, Internal Medicine 1, Department of Medicine (DIMED), AULSS2 Marca Trevigiana, Ca’ Foncello Hospital, University of Padova, 35124 Padova, Italy
Sarcoidosis and Granulomatous and Lymphocytic Interstitial Lung Diseases (GLILD) are two rare entities primarily characterised by the development of Interstitial Lung Disease (ILD) in the context of systemic immune dysregulation. These two conditions partially share the immunological background and pathologic findings, with granuloma as the main common feature. In this narrative review, we performed a careful comparison between sarcoidosis and GLILD, with an overview of their main similarities and differences, starting from a clinical perspective and ending with a deeper look at the immunopathogenesis and possible target therapies. Sarcoidosis occurs in immunocompetent individuals, whereas GLILD occurs in patients affected by common variable immunodeficiency (CVID). Moreover, peculiar extrapulmonary manifestations and radiological and histological features may help distinguish the two diseases. Despite that, common pathogenetic pathways have been suggested and both these disorders can cause progressive impairment of lung function and variable systemic granulomatous and non-granulomatous complications, leading to significant morbidity, reduced quality of life, and survival. Due to the rarity of these conditions and the extreme clinical variability, there are still many open questions concerning their pathogenesis, natural history, and optimal management. However, if studied in parallel, these two entities might benefit from each other, leading to a better understanding of their pathogenesis and to more tailored treatment approaches.
