Granulomatous disease: Distinguishing primary antibody disease from sarcoidosis

Chronic granulomatous disease associated with common variable immunodeficiency – 2 clinical cases

“…6,7 Granulomatous lesions in CVID may not only affect the same organs as sarcoidosis but also be histologically indistinguishable. 8---11 As a consequence, sarcoidosis is often incorrectly diagnosed in GD-CVID patients, with GD-CVID considered as a distinguished entity.…”

“…The patient's age and sex, diminished cellular immunity, the systemic involvement, and high levels of angiotensin-converting enzyme are common features for the two pathologies, so a previous story of recurrent/persistent bacterial infections should raise suspicion of CVID. 6,7 However, in a case---control study comparing GD-CVID patients and patients with pulmonary sarcoidosis, certain differences were detected, such as a random versus perilymphatic micronodular distribution on lung CT scan in GD-CVID patients, besides the higher frequency of consolidations with air bronchogram, bronchiectasis, or halo signs. Biological data showed that BAL CD4/CD8 ratio was significantly lower in GD-CVID.…”

“…However, both of them improved in clinical, analytical, and radiological terms, which is in line with the favorable evolution reported by Bouvry et al 1 The appropriate treatment for GD-CVID patients has not yet been established. 6,7,12,15 There are no protocols regarding diagnosis, treatment and follow-up, nor prognosis for GD-CVID patients. Many of these patients are followed individually and in a nonstandardized way.…”

“…All clinicians should be familiar with this pathology because so many organs and systems can be affected. 7,8,17 Ethical disclosures Protection of human and animal subjects. The authors declare that no experiments were performed on humans or animals for this study.…”

Common variable immunodeficiency

“…The term granulomatous and lymphocytic lung disease (granulomatous lymphocytic interstitial lung disease [GLILD]) has been used to characterize this disorder 30 and occurs in ϳ10-25% of patients with CVID. [29][30][31][32][33][34][35] Progressive pulmonary impairment due to GLILD appears to be an important cause of morbidity and mortality in CVID 30 and shows a shortened median survival (13.7 years versus 28.8 years in those without this complication). 1,12,30,36 This noncaseating granulomatous disease is systemic in nature and may be present in the lung, bone marrow, liver, and lymph nodes.…”