While confirming the already recognized risk factors, including cryoglobulin, splenomegaly, and sensorimotor neuropathy, we have, for the first time, identified male sex as being a risk factor for NHL in primary SS. Contrary to the comments made by Haacke et al, only 13% of patients with primary SS included in the study were male (consistent with previous studies), and not 50% as they reported. Half of the patients with NHL, however, were male. As discussed, we are unable to explain this high prevalence. However, we do believe that the association between male sex and NHL in primary SS is not coincidental or linked to recruitment or selection bias. As noted by Haacke and colleagues, the risk of lymphoma is associated with higher disease activity (1,2), consensually assessed by the ESSDAI score (2,3). We want to bring to their attention that recent analyses of the largest international primary SS cohort, which included 9,974 patients, showed that male patients with primary SS, compared to female patients, are characterized by higher disease activity at diagnosis. This includes a higher mean ESSDAI (8.0 versus 5.9; P < 0.001) and clinESSDAI (8.4 versus 6.1; P < 0.001), and clinically by a higher prevalence of lymphadenopathy (P < 0.001) and glandular involvement (P < 0.001) (4), both manifestations being recognized as predictors of NHL in primary SS.Haacke et al also pointed out the high prevalence of monoclonal gammopathy in patients with NHL. The prevalence of monoclonal gammopathy in our cohort (13%) was comparable to those previously reported (4-22%) (5). However, all 8 patients with NHL had monoclonal gammopathy, in contrast to approximately half of the patients in other studies. This may be explained by the presence of type II mixed cryoglobulin, which is characterized by the association of both a monoclonal gamma globulin component and polyclonal immunoglobulins in all patients.To conclude, we agree that the presence of ectopic GCs is associated with, and may reflect, more active disease (6). The time point when the biopsy is performed may be crucial for the detection and the significance of GCs in labial minor salivary glands. This is why we do not consider that the presence of GCs per se is only sufficient to determine all the risk for NHL in patients with primary SS.As reported in Tables 2 and 3 of our article, the other consensual risk factors for NHL, such as serum cryoglobulin, splenomega ly, parotid gland enlargement, sensorimotor neuropathy, low C4, and leukopenia, yielded higher predictive power than GCs. We agree with Haacke et al that the assessment of the risk of NHL should take into account all recognized clinical and laboratory risk factors, especially those that might be easily and regularly quantifiable throughout the patient's follow-up. All of the following parameters weighed heavily in the ESSDAI score: leukopenia, low C4 and C3 levels, rheumatoid factor positivity, the presence of cryoglobulin, purpura, sensorimotor neuropathy, lymphadenopathy, splenomegaly, and parotid enlargement (2,3).