Lymphopenia in Wegener’s Granulomatosis

Abstract

Background: Significant lymphopenia is a prominent feature in systemic erythematous lupus, but has not been described in Wegener’s granulomatosis (WG). We suggest from a retrospective analysis that lymphopenia may also be an index of WG disease activity. Methods: Medical charts form 19 patients diagnosed with systemic active WG between 1990 and 2000 were reviewed retrospectively. All patients had crescentic glomerulonephritis and alveolar hemorrhage. Clinical and biological markers were reviewed at three different time points: diagnosis, time of relapse, and during remission. Results: Average lymphocyte count was significantly lower at diagnosis and relapse than during remission times (p < 0.008 and p < 0.000002, respectively). During disease activity either at diagnosis and during relapses, ANCA titers were highly positive (≧50 IU/ml) in 27.5% of patients (8/29). The corresponding lymphocyte counts were below normal (1,500/min³) in 28 of 29 patients (96.5%). In patients with either negative or weakly positive ANCA, mean lymphocyte count was 728.5, 744.2 and 2,551/ml at diagnosis, during relapse and remission times, respectively. There was a clear negative correlation between the lymphocyte count and disease activity. Conclusion: Lymphopenia appears to be a good marker of WG activity. This index might be useful in all patients including those with negative ANCA.

Impaired phagocytosis and reactive oxygen species production in phagocytes is associated with systemic vasculitis

“…In line with earlier observations, we found that patients with AAV had a decreased percentage of lymphocytes and an increase of PMN. Low levels of lymphocytes are common on diagnosis of AAV and during flares [ 33 ] and might also be an effect of immune suppressive treatment [ 34 , 35 ]. The increased percentage of PMN in patients with AAV could be explained by increased survival in the periphery in combination with elevated bone marrow release.…”

Lymphocyte counts in patients with ANCA-associated vasculitis

“…One study reported that treatment-induced lymphopenia (·0.5 £ 10 9 /L) is associated with a high likelihood to achieve sustained remission [3,7]. By contrast, another group suggested that, analogous to patients with systemic lupus erythematodes, lymphopenia in AAV reXects disease activity, with lymphocyte counts actually increasing in patients achieving remission [8,9]. While the reason for these contradictory Wndings remain unclear, neither study has taken into account short-term variability of lymphocyte counts and how this might impact study results.…”