>Corresponding Author : MOUSSAIF J
>Article Type : Case Report
>Volume : 6 | Issue : 3
>Received Date : 20 August 2026
>Accepted Date : 28 August 2026
>Published Date : 31 August 2026
>DOI : https://doi.org/10.54289/JCRMH2600112
>Citation : MOUSSAIF J, ETAOUAS C, EL hachami FZA, Madyani H, Assal A, et al. (2026) Still’S Disease and Pregnancy: a Case Report and Review of the Literature. J Case Rep Med Hist 6(3). doi: https://doi.org/10.54289/JCRMH2600112
>Copyright : © 2026 MOUSSAIF J, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Case Report | Open Access | Full Text
1Gynecology obstetrics residents at CHU Ibn Rochd Casablanca, Morocco
2
Professors of Gynecology obstetricsat : Gynecology Department A, CHU Ibn Rochd Casablanca, Morocco
*Corresponding author: MOUSSAIF J, Professors of Gynecology obstetricsat : Gynecology Department A, CHU Ibn Rochd Casablanca, Morocco
Adult-onset Still’s disease (AOSD) is a rare systemic disorder of unknown etiology and pathogenesis [1,2], with an estimated prevalence ranging from one to 34 cases per million inhabitants [3], and affecting predominantly young women [4,5].
Yamaguchi’s criteria [6] are commonly used, as they suggest the main clinical symptoms and laboratory findings of this
disease. Typical manifestations include a remittent fever above 39 °C lasting more than one week, arthralgia or arthritis,
the characteristic rash, leukocytosis, elevated liver enzymes, and splenomegaly. In addition, some cases of AOSD may present with abdominal pain and nausea [2]. If such findings as elevated liver enzymes and abdominal pain occur during pregnancy, AOSD may be misdiagnosed as hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome or
acute fatty liver of pregnancy (AFLP). To date, although the association between disease onset and pregnancy has been extensively studied [7,8], this relationship remains unclear. Moreover, only a few cases of AOSD flare-ups during the third
trimester have been reported, and neither the management nor the features of this condition, which resembles HELLP syndrome and AFLP, during late pregnancy have yet been described. First-line treatment consists of high-dose corticosteroid
administration [2,9,10]. However, some cases are refractory to corticosteroids, and Tocilizumab (TCZ), an interleukin-6 inhibitor, has proven effective in such situations [11]. At present, the relationship between pregnancy and AOSD, including
disease onset and relapse, remains uncertain. Only a limited number of studies have been published, mainly in the form of
case reports or small case series, and therefore no conclusive interpretation is available [8,12-14]. Here, we report the rare
case of a 5-month-pregnant woman with Still’s disease, who had recurrent thrombocytopenia since the first trimester, and
we review previous reports on pregnancy-related AOSD flares. The patient provided written informed consent for publication of this case report.
Keywords: Still, Pregnancy, Autoimmune, Treatment
Abbreviations: AFLP: Acute fatty liver of pregnancy; AOSD: Adult onset Still’s disease; HELLP: Hemolysis, elevated
liver enzymes, and low platelet count; IVIG: Immunoglobulin; TCZ: Tocilizumab
A 27-year-old primigravida, primiparous woman with a history of Still’s disease for two years, followed up in the Department of Internal Medicine, had been hospitalized during the
first trimester for severe thrombocytopenia with hemorrhagic
manifestations and was started on corticosteroid therapy. The
patient, however, discontinued treatment on her own. She was
later admitted to the maternity ward for threatened preterm labor associated with severe thrombocytopenia at an estimated
gestational age of 22 weeks.
On admission, clinical examination found a conscious patient
with a Glasgow Coma Scale score of 15/15, in good general
condition, blood pressure 110/70 mmHg, respiratory rate 18
cycles/min, heart rate 80 beats/min, negative urine dipstick,
capillary blood glucose 0.99 g/dL, and afebrile at 36.5 °C. She
presented with widespread maculopapular lesions on the chest
and ecchymotic patches on the lower limbs. Uterine contractions were noted, with a cervix dilated to fingertip size and intact membranes.
Obstetric ultrasound revealed an ongoing singleton pregnancy
with fetal biometrics consistent with gestational age, a fundal
placenta, shortened cervical length of 21 mm, and a large cisterna magna measuring 50 mm.
Laboratory investigations showed hemoglobin 12.3 g/dL,
leukocytes 15,460/mm³, and severe thrombocytopenia at
13,000/mm³, confirmed on citrate tube and smear review.
Other tests were unremarkable, including coagulation profile,
renal and liver function. Urine culture and vaginal swab were
sterile. The 24-hour proteinuria was negative, and infectious
serologies were negative. Bone marrow aspiration revealed thrombocytopenia of peripheral origin with inflammatory features. Immunological work-up was negative, including protein
S, activated protein C resistance, antithrombin, and antiphospholipid antibodies.
The patient was treated with corticosteroids and azathioprine,
with improvement in both clinical symptoms and thrombocytopenia. She delivered at 39 weeks of gestation by cesarean
section a live male newborn, Apgar 9/10 and 10/10, birth
weight 2900 g, with no evidence of thrombocytopenia on complete blood count.
In the postpartum period, the patient maintained platelet
counts between 40,000/mm³ and 100,000/mm³ over six
months of follow-up.
A flare of adult-onset Still’s disease (AOSD) during pregnancy was first reported in 1980 by Stein et al [15]. The relationship between the development of AOSD and pregnancy remains unclear. Moreover, cases of AOSD with onset during
the perinatal period are rare. The clinical course of AOSD during pregnancy has been classified into three types: the de novo
type, referring to first onset of the disease in relation to pregnancy; the recurrent flare type, referring to pregnancy-related
recurrence in patients with a previous diagnosis of AOSD; and
the non-flare type, referring only to complications without
pregnancy related flare [7,8,15]. In the literature, reported cases have been divided into these three types, and our case belongs to the second type.
The onset of AOSD during pregnancy remains rare [15] but
can compromise maternal and fetal outcomes, including
preterm delivery [14]. It has been noted that the gestational
age at onset is typically in the first or second trimester (8 to 26
weeks of amenorrhea) according to published reviews [14].
The median maternal age at pregnancy was reported to be 30
years [16]. With respect to parity, AOSD flares occur mainly
in primiparous women [8], as was the case in our patient.
AOSD is highly heterogeneous in its clinical manifestations
[17]. Fever is considered the cardinal feature, followed by
arthritis, while rash represents the third cardinal sign. Typically, the cutaneous eruption consists of small, salmon-pink macules or maculopapules, non-pruritic, often localized to the
proximal limbs, trunk, and occasionally the palms and soles
[8,17]. Other clinical manifestations may include arthralgia,
neurological or ophthalmic involvement. In our case, the patient presented with cutaneous rash on the trunk and lower
limbs.
There is no specific biological marker of AOSD. However,
two laboratory features are highly suggestive: neutrophilic leukocytosis and markedly elevated serum ferritin, often with a
low glycosylated ferritin fraction. Another supportive finding
is the absence of autoimmunity, particularly negative rheumatoid factor and antinuclear antibodies. In contrast, abnormal
liver function tests are found in up to two-thirds of patients
[17]. Given the wide range of clinical presentations—from isolated prolonged fever to neoplastic mimicking conditions—the diagnosis of AOSD is often complex and sometimes delayed.
In 2004, Mok et al. reported new findings in 22 pregnancies in
17 women [13]. Disease flares were observed in 19 pregnancies, mostly during the fifth and sixth months and in the postpartum period. In 2012, Yamamoto et al. described 25 pregnancies in 19 women [18], with at least one flare observed in
most cases. Disease exacerbations occurred primarily in the second trimester and postpartum. A favorable course was noted
in only six pregnancies. The main complications included spontaneous abortion, intrauterine growth restriction (IUGR),
preterm delivery, and one neonatal death. Maternal complications were rare but included gestational diabetes in one patient, preeclampsia in another, and macrophage activation syndrome in two cases.
A 2018 retrospective, descriptive, and analytical study [19],
conducted at the Internal Medicine Department of Libreville
University Hospital, reviewed medical records of patients
with autoimmune diseases including AOSD and pregnancy, regardless of outcome. Reported complications included two
spontaneous miscarriages in the first trimester (n=1), two intrauterine deaths, one perinatal death at day 12, and two cases
of eclampsia, one complicated by pulmonary embolism during
a first pregnancy. The mean gestational age at delivery was 37
weeks, with most cases delivering beyond 35 weeks (n=18)
compared to fewer cases before 35 weeks (n=9). Intrauterine
growth restriction was observed in 10 cases, and preterm delivery in 17 cases.
In our case, the patient received corticosteroids and azathioprine, with improvement in clinical signs and thrombocytopenia. She delivered at 39 weeks of gestation, and during six
months of postpartum follow-up, her platelet count remained
between 40,000/mm³ and 100,000/mm³.
Based on these series and our case, pregnancy appears to favor AOSD flares, particularly at the end of the first and second trimesters and in the postpartum period. Treatment of
AOSD during pregnancy can be challenging. Corticosteroids
are commonly used but carry risks such as gestational diabetes, hypertension, and the need for close monitoring. Intravenous immunoglobulin (IVIG) may be considered in life-threatening cases [20]. Azathioprine is sometimes preferred as a
steroid-sparing agent [21].
Reported cases during pregnancy remain limited, and current
therapeutic experience is largely derived from case reports
and small series.
In summary, we found that pregnancy in patients diagnosed with AOSD was associated with an increased risk of complicated pregnancies, which should be anticipated by rheumatologists, internists, and obstetricians alike. However, AOSD activity was not exacerbated by pregnancy when the disease was well controlled, highlighting the importance of disease assessment prior to conception.