Table 3 Autoantibodies profile in various series
Group | ANCA n (%) | PR3 n (%) | MPO n(%) | RF n(%) | Anti CCP n(%) | ANA n(%) | ENA n(%) | Treatment experience |
|---|---|---|---|---|---|---|---|---|
Watkin LB. et al., 2015 [3] (n = 21) | 15 (71) | NA | NA | 9 (43) | NA | 14 (67). | 0/0 (100%) | Symptomatic partial response to immunosuppression 21 (100) |
Jensson BO.]et al. 2017 [4] (n = 3) | 2 (67) | NA | NA | 3 (100) | 1 (33%) | 3 (100) Titers up to 1:1280 | NA | Partial response to immunosuppression Responded to MMF, Hydroxychloroquine, and steroids. |
Brennan MA. et al.2017 [8] (n = 1) | NA | NA | NA | NA | NA | 1/1 (100%) | 1/1 (100%) | Pulse and oral steroids, Monthly-six doses of Cyclophosphamide, MTX, Rituximab. Currently on combination therapy: MMF, Hydroxychloroquine, steroids. Comorbidities: GERD, Cystic Fibrosis, SLE. Developed MAS. |
Volpi S. et.al. 2018 [7] (n = 1) | 0/1 (0%) | NA | NA | 1/1 (100%) | NA | Yes (but not at the presentation) | NA | Partial response to immunosuppression |
Tsui JL. et.al. 2018 [2] (n = 14) | 9/12 (64%) | 3/9 (33%) | 2/9 (22%) | 10/14 (71%) | 3/14 (21%) | 12/14 (86%) titers 1:40 to 1:320 | 0/14 (100%) | Partial response to immunosuppression Most patients with diffuse alveolar hemorrhage (DAH) responded to pulse methylprednisolone and monthly-six doses of cyclophosphamide, but few subsequently needed rituximab. Poor response to Methotrexate, AZA, and Etanercept. Most patients are doing better on low dose prednisone+ MMF+ Hydroxychloroquine. 2/14 (14%) needed a bilateral lung transplant. |
Patwardhan A. et.al. (n = 2) | 2/2 (100%) | Index case: Negative Father of the index case: NA | Index case: Negative Father of the index case: NA | 0/2 (0%) | Index case: Negative Father of the index case: NA | 2/2 (100%) Titers up to 1:1280 | Index case: Negative Father of the index case: Negative | IC had a partial response to immunosuppression (no response to Methotrexate, AZA). Good response to pulse steroids followed by MMF+ low dose oral steroids. C2-was treated with repeated pulse steroids, oral high and low dose steroids and now with adalimumab and methotrexate for arthritis. |