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Skin and muscle involvement: (a) rash on her face and (b) bilateral diffuse inflammation in thigh muscles (magnetic resonance imaging).
Source publication
Dermatomyositis (DM), a subtype of idiopathic inflammatory myopathies (IIMs), is characterized by skin rash, proximal muscle weakness, and inflammatory infiltrates in the muscle tissue. The peak incidence of the disease is at the age of 50–60 years, and only 14% of the patients with IIMs are estimated to present during reproductive years. Because o...
Citations
... In adults, dermatomyositis commonly emerges in the late 40s to early 60s. In children, it manifests between the age group of five and 15 and demonstrates a higher occurrence in females than males [1]. Dermatomyositis displays symmetric, proximal greater than distal muscle weakness along with a characteristic rash that includes the heliotrope rash, Gottron papules (raised erythematous rash over knuckles), V-sign, holster sign, shawl sign over the back of the neck and shoulder, nail bed telangiectasias, and subcutaneous calcium deposits [1]. ...
... In children, it manifests between the age group of five and 15 and demonstrates a higher occurrence in females than males [1]. Dermatomyositis displays symmetric, proximal greater than distal muscle weakness along with a characteristic rash that includes the heliotrope rash, Gottron papules (raised erythematous rash over knuckles), V-sign, holster sign, shawl sign over the back of the neck and shoulder, nail bed telangiectasias, and subcutaneous calcium deposits [1]. ...
Dermatomyositis represents a rare inflammatory myopathy that induces inflammation in the muscles or related tissues, including the blood vessels supplying these muscles. The precise pathogenesis of this condition remains unknown. Diagnosis typically relies on clinical indicators such as skin rashes, progressive muscle weakness, elevated serum muscle enzymes, abnormal electromyogram results, and muscle biopsy. In this case study, we report a fatal case of dermatomyositis in a 23-year-old female patient who succumbed to complications of dermatomyositis, causing mortality without any evidence of malignancy.
... For muscle or lung involvement or refractory cutaneous disease, low-dose corticosteroids should be initiated along with azathioprine to achieve a maintenance corticosteroid dose below 20 mg of prednisone daily (29, 30, 48-50, 53, 54). In severe instances, intravenous immunoglobulin (IVIG) can be used as limited research suggests its relative safety during pregnancy (48,54,57). IVIG notably crosses the placenta significantly only after the 32 nd week of gestation and is also compatible with breastfeeding (29,30,40,49,54,62). ...
Introduction
Dermatomyositis, systemic and cutaneous lupus erythematosus have a significantly higher prevalence in women than men, emphasizing the relevance of exploring the relationship between sex hormones and autoimmune skin diseases. This review analyzes the interplay between sex hormones and these two skin diseases.
Materials and methods
We performed an extensive literature search using the PubMed database from July to August 2023. Search terms included ‘contraceptives’, ‘pregnancy’, ‘hormone replacement’, ‘tamoxifen’, and ‘aromatase inhibitors’.
Results and Discussion
This comprehensive literature review shows that there remains considerable debate regarding the use of hormonal contraceptives and hormonal replacement therapy in individuals with autoimmune skin conditions. Nonetheless, it is well established that their use is contraindicated in patients with antiphospholipid syndrome or when antiphospholipid antibodies are positive. Individuals experiencing disease flares and uncontrolled symptoms should also avoid these interventions. Pregnancy planning should be timed to coincide with well-managed disease states to minimize obstetric and neonatal complications. Hormonal breast cancer treatment requires close skin monitoring.
Conclusion
Pregnancy, menopause, contraceptive use, hormone replacement therapy, and breast cancer treatment drugs result in substantial shifts in hormone levels. Additionally, hormone levels are altered by aromatase inhibitors and anti-estrogen medications. These fluctuations can modulate mechanisms influencing autoimmune skin abnormalities.
... It was reported that pregnancy during disease activity or the onset of DM/PM during pregnancy leads to poor foetal prognosis [6,22]. Munira et al. provided an overview of pregnancy outcomes in DM/PM and concluded that adequate control of disease activity is critical for the foetal outcome because the active disease is associated with poor foetal prognosis [29]. ...
Although pregnancy is an important risk factor for autoimmune rheumatic diseases, little is known regarding the association between pregnancy and dermatomyositis (DM) or polymyositis (PM). Herein, we present two patients with DM that developed during the perinatal period. The first patient was positive for anti-aminoacyl synthetase (ARS) antibody and developed DM in the 14th week of pregnancy. Despite treatment, her foetus died of intrauterine growth restriction in the 27th week. The second patient was positive for anti-melanoma differentiation-associated gene 5 (MDA-5) antibody and developed DM 1 week after miscarriage at 9 weeks of gestation. The patient developed severe interstitial pneumonia, and intensive therapy including tofacitinib and rituximab administration was required. Our cases and a literature review revealed that various myositis-specific autoantibodies, including anti-ARS, anti-Mi-2, anti-TIF-1γ, and anti-MDA-5, are associated with DM and PM triggered by pregnancy. We also found that delay in commencing treatment in case of active disease including myositis and interstitial pneumonia, and poor response to corticosteroids were related to poor foetal outcomes in DM and PM. Although rare in pregnant women, it is critical to consider the possibility of DM and PM in patients presenting with rash, fever, weakness, and cough, and testing for myositis-specific autoantibodies is recommended.
... Treatment regimen should consider the safety of both the mother and the fetus, which requires individualized therapy. Glucocorticoids are the first line treatment in pregnant patients with DM (24). In certain rare cases, the use of glucocorticoids has been demonstrated to lead to a good outcome (6). ...
... In certain rare cases, the use of glucocorticoids has been demonstrated to lead to a good outcome (6). However, certain patients have been shown to be non-responsive or intolerant to glucocorticoids (24). A previous study illustrated that gestational exposure to glucocorticoids led to a slight increase in the risk of premature birth and fetal oral cleft (25). ...
... A previous study illustrated that gestational exposure to glucocorticoids led to a slight increase in the risk of premature birth and fetal oral cleft (25). Efficacy and safety of IVIG during pregnancy has been well documented in DM, especially for refractory cases (24,25). In a previously published case report, short term remission was achieved following treatment with IVIG (4.5 g for 3 consecutive days) (25). ...
Dermatomyositis occurs extremely rarely during pregnancy. A number of studies in the published literature have documented how the outcome of pregnancy is poor for both mother and fetus. The present case study reports on a patient who was diagnosed with clinically amyopathic dermatomyositis complicated by interstitial lung disease during pregnancy, and was successfully treated with a combined immunosuppressant regimen. To the best of the authors’ knowledge, this is the first case study detailing how a pregnant woman with clinically amyopathic dermatomyositis with positive anti-melanoma differentiation-associated gene 5 antibody achieved complete remission after early intervention of combined immunosuppressive therapy without residual pulmonary interstitial changes.
... Of the 801 births in women with IIM, 68 were post-IIM and 736 were pre-IIM, corresponding to 236 and 3865 non-IIM comparators, respectively. The median (IQR) age at IIM diagnosis and disease duration at delivery of post-IIM births was 28 (15)(16)(17)(18)(19)(20)(21)(22)(23)(24)(25)(26)(27)(28)(29) years and 4.43 (1.69-9.48) years, respectively. ...
... Yet, the risks might be overestimated as the study required hospitalization for IIM, which might lead to selection of women with high disease activity/severity in that study. In several small scale clinic-based studies and in a review, higher risk of preterm birth was associated with active disease status [2,4,5,27]. We identified women with IIM from both inpatient and outpatient clinics, and therefore disease activity may have varied from mild to severe, which might explain the difference between our findings and the Australian study although our study did not find any different risk estimates among births to women with IIM identified from the inpatient vs the outpatient register. ...
Objectives:
To examine pregnancy outcomes among births to women with idiopathic inflammatory myopathy (IIM) in relation to time of IIM diagnosis using population-based data.
Methods:
This study used Swedish nationwide registers to identify all singleton births that occurred between 1973 and 2016 among women diagnosed with IIM between 1998 and 2016 and among women unexposed to IIM. We classified births according to the IIM status of the mother at time of delivery: post-IIM (n = 68), 1-3 years pre-IIM (n = 23), >3 years pre-IIM (n = 710) and unexposed to IIM (n = 4101). Multivariate regression models were used to estimate relative risks of adverse pregnancy outcomes in post-IIM births and pre-IIM births separately, in comparison with their non-IIM comparators.
Results:
We found that post-IIM births had increased risks of caesarean section [adjusted relative risk (aRR) = 1.98; 95% CI: 1.08, 3.64], preterm birth (aRR = 3.35; 95% CI: 1.28, 8.73) and low birth weight (aRR = 5.69; 95% CI: 1.84, 17.55) compared with non-IIM comparators. We also noticed higher frequencies of caesarean section and instrumental delivery in 1-3 years pre-IIM births than in the non-IIM comparators.
Conclusion:
Women who gave birth after IIM diagnosis had higher risks of caesarean section, preterm birth and low birth weight. These results further underline the importance of special care and close monitoring of women with IIM. Higher frequencies of caesarean section and instrumental delivery in pre-IIM births highlight the need for future research on the influence of subclinical features of IIM on pregnancy outcomes.
... Katz [2] and Morihara et al. [3] have described a relationship between autoimmune myositis (myopathy) and pregnancy as attributable to changes in the mother's hormones or the fetus being recognized as a foreign body. Missumi et al. [4] reported in their case series covering 15 cases that the disease activity of myositis does not increase because of pregnancy; however, several other reports have associated the disease activity of myositis with fetal prognosis, and it has, moreover, been associated with higher rates of preeclampsia, preterm birth, or fetal death [5][6][7]. According to these reports, favorable control of disease activity before or during pregnancy would likely improve the prognosis of both the mother and child in pregnancies complicated with autoimmune myositis (myopathy). ...
Polymyositis-dermatomyositis is extremely rare during pregnancy, and immunosuppressive therapy should be administered after carefully considering the effects on both the mother and fetus. Several reports have associated the disease activity with fetal prognosis, higher rates of eclampsia, preterm births, and fetal deaths. We report our experience with a patient who was diagnosed with polymyositis-dermatomyositis complicated by interstitial lung disease during pregnancy and was treated with a combination-immunosuppressant regimen. To the best of our knowledge, this is the first case wherein cyclosporine was used concomitantly with a steroid for the treatment of polymyositis diagnosed during pregnancy, with successful outcome of childbirth without any complications.
... Patients with well controlled or inactive disease during pregnancy have good pregnancy outcomes for both the mother and fetus, [112][113][114] while active disease during pregnancy appears to be associated with a high risk of premature birth or spontaneous abortion. 115,116 CS are the mainstay of disease management for DM/PM in pregnancy, though IVIG has been successfully used. 117,118 Conclusion Neuromuscular disorders may first present during pregnancy or the postpartum period. ...
Neuromuscular disorders may present and progress differently in women than in men. During pregnancy, medication adjustment, hormonal effects, and other alterations in physiology may influence the manifestation of a variety of neuromuscular disorders. The expression of existing conditions may change; previously asymptomatic conditions may be unmasked, or entirely new conditions may develop. Additionally, neuromuscular disorders and their treatments may have implications for the fetus. Such factors must be carefully considered when counseling and treating pregnant women and those considering pregnancy. This article reviews considerations specific to women and issues surrounding pregnancy in disorders of the neuromuscular junction, focal neuropathies, and acquired and inherited disorders of the nerve and muscle.
Objectives
Pregnancy outcomes in women with inflammatory myopathies (IM) are not well studied. The purpose of this study is to evaluate the effects of IM on maternal and neonatal outcomes.
Methods
We conducted a retrospective cohort study using data from the Healthcare Cost and Utilization Project – Nationwide Inpatient Sample (HCUP-NIS) from 1999 to 2015. Among all pregnant women who delivered during this period, those with a diagnosis of IM were identified using the International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) coding, which included all patients with dermatomyositis and polymyositis. Maternal and neonatal outcomes were compared in pregnant women with and without IM. Multivariate logistic regression analysis was used to estimate the adjusted effects of IM on these outcomes.
Results
A total of 13,792,544 pregnant women delivered between 1999 and 2015, of which 308 had a diagnosis of IM, for an overall prevalence of 2 per 100,000 pregnant women, with rates increasing over the study period. Pregnant women with IM were more likely to be older, African American and suffer from other autoimmune connective tissue diseases. IM in pregnancy was associated with greater risk of preeclampsia, caesarean delivery, major postpartum infections, urinary tract infections and longer hospital stay. Neonates born to mothers with IM had greater risk of prematurity, small for gestational age and intrauterine fetal demise.
Conclusions
Pregnant women with IM are at higher risk of adverse maternal and neonatal outcomes and should be closely followed in specialized centers with collaboration between maternal-fetal medicine and rheumatology.
We encountered a 30-year-old woman who developed dermatomyositis during pregnancy and was positive for anti-Mi-2 antibodies. She was successfully treated with prednisolone and tacrolimus and delivered a healthy child. We reviewed the cases of idiopathic inflammatory myositis (IIM) that developed during pregnancy that were published after the year 2000 to elucidate the profile of myositis-specific antibodies (MSAs) in them and to evaluate their obstetric outcomes. In cases with IIM that developed during pregnancy, anti-Mi-2, anti-TIF1-g, anti-Jo-1, and anti-EJ antibodies was detected in one case each. The obstetric outcomes of the IIM-complicated pregnancies were poor, especially when complicated with active maternal myositis. Further studies focusing on the possible causal relationships between MSAs and cases with IIM that developed during pregnancy are needed. For better obstetric outcomes, appropriate suppression of the maternal disease activity using immunosuppressants and vigilance regarding the patient's requirement of Caesarean section is important.
