Granulomatosis-associated common variable immunodeficiency disorder: a case-control study versus sarcoidosis

Abstract

The aim of the present study was to investigate to what extent interstitial lung disease (ILD) in common variable immunodeficiency disorder (CVID)-associated granulomatous disease (GD) is similar to pulmonary sarcoidosis 20 patients with CVID/GD were included in a retrospective study conducted by the Groupe Sarcoïdose Francophone. Medical records were centralised. Patients were compared with 60 controls with sarcoidosis.Clinical examination showed more frequent crackles in patients than controls (45% versus 1.7%, respectively; p,0.001). On thoracic computed tomography scans, nodules (often multiple and with smooth margins), air bronchograms and halo signs were more frequent in patients than controls (80% versus 42%, respectively; p50.004) as well as bronchiectasis (65% versus 23%, respectively; p,0.001). The micronodule distribution was perilymphatic in 100% of controls and in 42% of patients (p,0.001). Bronchoalveolar lavage analysis showed lower T-cell CD4/CD8 ratios in patients than in controls (mean¡SD 1.6¡1.1 versus 5.3¡4, respectively; p,0.01). On pathological analysis, nodules and consolidations corresponded to granulomatous lesions with or without lymphocytic disorders in most cases. Mortality was higher in patients than controls (30% versus 0%, respectively) and resulted from common variable immunodeficiency complications.ILD in CVID/GD presents a specific clinical picture and evolution that are markedly different from those of sarcoidosis.

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

“…The second patient did not receive any therapy directed at the GD. 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.…”

“…1 It is defined by markedly reduced serum concentrations of IgG, combined with low levels of IgA and/or IgM, poor or absent response to immunizations in a patient without any other signs of immunodeficiency. The prevalence in Europe is estimated to be around 1:50,000---1:200,000.…”

“…However Bouvry et al in their case---control study did not find GD to be a critical determinant of prognosis but found a worse outcome to be linked to the classical complications of CVID such as lymphoma, infection or obstructive lung disease secondary to bronchiectasis. 1 The clinical presentations of the two reported clinical cases, asymptomatic mediastinal and hilar adenopathies and erythema nodosum, are among the most frequent presentations of sarcoidosis. In addition CVID was already known in both patients; however GD-CVID can, although rarely, be the presentation of the disease.…”

Bronchoalveolar lavage cytology resembles sarcoidosis in a subgroup of granulomatous CVID

“…We read with great interest the article by BOUVRY et al [1] regarding similarities and differences between interstitial lung disease (ILD) in granulomatous common variable immunodeficiency (CVID) and sarcoidosis. In this retrospective study, differential bronchoalveolar lavage (BAL) cytology was analysed in 14 patients with granulomatous CVID and ILD.…”

“…Unlike the sarcoidosis group (5.3¡4.0), the CD4/CD8 ratio was low in the analysed patients with granulomatous CVID and ILD (1.6¡1.1, n510) and even ,1 in half of the patients (n55). BOUVRY et al [1] concluded that there are significant differences in differential BAL cytology between sarcoidosis and granulomatous CVID.…”