Table 2. Clinical characteristics, treatment modalities, and obstetric outcomes in pregnant patients with adult-onset Still's disease onset in pregnancy: a case series overview.
Case | Source reference | Age at Dx | Parity | Disease onset | Clinical presentations | Treatment | MOD | GA at delivery (wk) | Adverse obstetrical outcomes | Disease course | Postpartum |
---|---|---|---|---|---|---|---|---|---|---|---|
A01 | Kaplinsky et al (1980) 42 | 34 | G5P4 | Unk | F, PA, R, SM, ↑ WBC | Gold | Unk | Unk | None | Polycyclic | Worsening of disease |
A02 | Green et al (1982) 43 | 23 | Unk | Second tri | F, A, R, HM, SM | PDN | Unk | 28 | PTD, neonatal death due to prematurity | Polycyclic | Worsening of disease |
A03 | Yebra Bango et al (1985) 44 | 19 | Unk | First tri | A, anorexia, HM, R, SM, ST | PDN | VD | Unk | None | Monocyclic | No flare-ups |
A04 | Le Loët et al (1993) 45 | 27 | Unk | Second tri | A, F, LAD, pharyngitis, R | PDN | Unk | Unk | Transient Olig | Polycyclic | Unk |
A05 | Le Loët et al (1993) 45 | 30 | Unk | First tri | Unk | PDN | Unk | Unk | Unk | Unk | Unk |
A06 | Le Loët et al (1993) 45 | 24 | Unk | Second tri | A, F, LAD, pharyngitis, myalgias, R | PDN | VD | Unk | None | Polycyclic | Unk |
A07 | Falkenbach et al (1994) 46 | 25 | G1P0 | First tri | A, F, headache, myalgias, LAD, SM, HM, ST, ↑ WBC | IVIG, PDN | N/A | N/A | ETOP | Polycyclic | Unk |
A08 | Liozon et al (1999) 47 | 28 | G1P0 | First tri | F, LAD, PA, R, ST, ↑ WBC, ↑ Ferritin, ↑ AST/ALT | Salicylates 3 g/24 h, IVIG, PDN | C/S | 36 | PEC, placenta abruption, PTD | Polycyclic | Flare at 5 mo |
A09 | Pan et al (2003) 48 | 21 | G2P1 | Second tri | F, A, R, malaise, cough, headaches, myalgias, odynophagia, ↑ AST&ALT, ↑ Ferritin | PDN, HCQ | VD | 34 | IUGR (IOL) | Polycyclic | Persistence of symptoms |
A10 | Pan et al (2003) 48 | 38 | G3P1 | Second tri | F, A, malaise, cough, ST, ↑ ferritin | PDN | VD | FT | None | Monocyclic | No flare-ups |
A11 | Fischer-Betz et al (2011) 25 | 29 | G2P1 | First tri | F, PA, HM, pharyngitis, myalgias, SM, R, ↑WBC, ↑ Ferritin | PDN, anakinra | C/S | 36 | PTD | Polycyclic | Flare after few months |
A12 | Yamamoto et al (2012) 49 | 28 | G0P0 | Second tri | F, ST, LAD, R, PA, synovitis, pleural effusion, ↑ ferritin, ↑ WBC, ↑ AST and ALT | PSL, plasma exchange, cyclosporine, dexamethasone | C/S | 33 | HLH, oral ulcers induced by CMV, IUGR | Monocyclic | No flare-ups |
A13 | Hammami et al (2013) 50 | 32 | G1P0 | Second tri | F, PA, R, SM, weight loss, ↑ ferritin | PDN | VD | 34 | PTD | Monocyclic | No flare-ups |
A14 | Gerfaud-Valentin et al (2014) 51 | 33 | Unk | First tri | F, erythema, LAD, PA, ↑ WBC | PDN | Unk | 34 | PPROM | Polycyclic | Flare at 1 mo |
A15 | Gerfaud-Valentin et al (2014) 51 | 27 | Unk | Second tri | F, PA, erythema, SM | PDN + IVIG + NSAIDs | Unk | 32 | PTD, oligo | Polycyclic | Flare at 1 and 3 mo |
A16 | Gerfaud-Valentin et al (2014) 51 | 36 | Unk | Second tri | F, PA, erythema, pharyngitis, LAD, SM | PDN | Unk | FT | None | Monocyclic | Unk |
A17 | Moussa and Hassan (2014) 52 | 25 | G1P0 | Second tri | F, A, malaise, HM, SM, ↑ WBC, ↑ ferritin | PDN | Unk | Unk | Unk | Unk | Unk |
A18 | Odai et al (2015) 53 | 32 | Unk | Second tri | F, R, LAD, PA, ST, ↑ WBC, ↑ ferritin | PSL, LCAP twice weekly × 12 wk | C/S | 34 | PPROM | Monocyclic | No flare-ups |
A19 | Lin et al (2016) 16 | Unk | Unk | Third tri | F, A, HM, SM, R, LAD, TTP | Unk | Unk | Unk | PTD, neonatal HLH | Unk | Unk |
A20 | Nakamura et al (2016) 28 | 22 | G1P0 | Second tri | F, R, PA, ↑WBC, ↑ferritin | PSL, tacrolimus | Unk | 38 | None | Monocyclic | No flare-ups |
A21 | Plaçais et al (2018) 54 | 38 | Unk | First tri | F, myalgias, PA, HM, ST, ↑ ferritin, ↑ AST and ALT | PDN, colchicine, IVIG | Unk | 34 | Acute agranulocytosis (related to colchicine) c/b severe sepsis | Polycyclic | Disease persisted despite delivery and sepsis control; addition of anakinra allowed rapid remission |
A22 | Present case | 29 | G3P2 | Second tri | F, headaches, vomiting, ↑AST and ALT | Methylprednisolone, azathioprine, anakinra | C/S | 32 | HLH, acute agranulocytosis (related to anakinra) c/b severe sepsis, placental abruption, GDM | Polycyclic | No flare-ups |
Abbreviations: A, arthralgia/arthritis; ALT, alanine aminotransferase; AST, aspartate aminotransferase; C/S, cesarean section; CMV, cytomegalovirus; CS, corticosteroids; Dx, diagnosis; ETOP, elective termination of pregnancy; F, fever; FT, full-term; GDM, gestational diabetes mellitus; HCQ, hydroxychloroquine; HLH, hemophagocytic lymphohistiocytosis; HM, hepatomegaly; IOL, induction of labor; IUFD, intrauterine fetal demise; IVIG, intravenous immunoglobulin; LAD, lymphadenopathy; LCAP, leukocytapheresis; MOD, mode of delivery; N/A, not applicable; NSAIDs, nonsteroidal anti-inflammatory drugs; Olig, oligohydramnios; PA, polyarthralgia/polyarthritis; PDN, prednisone; PSL, prednisolone; PTB, preterm birth; PTD, preterm delivery; R, rash; SM, splenomegaly; ST, sore throat; tri, trimester; Unk, unknown; VD, vaginal delivery; WBC, white blood cell count.