ReviewPregnancy and vasculitis: A systematic review of the literature
Introduction
Primary systemic vasculitis are uncommon diseases characterized by a great variety of symptoms, ranging from mild to life threatening manifestations and encompassing different natural histories, from self-limiting to relapsing or chronic active disease.
Over the past years, the number of pregnancies among affected women greatly increased, due to improvement in survival as well as in quality of life of these patients. Frequency of pregnancy in different vasculitis depends on epidemiological factors: pregnancy is more frequent in vasculitis that have onset at younger age and affect the female gender, such as Takayasu's arteritis and Behçet disease.
During pregnancy, immune and endocrine systems undergo profound transformations concerning both hormonal assessment and cytokine microenvironment: cortisol, progesterone, estradiol and testosterone increase physiologically during gestation and seem to favor Th-2 cytokine polarization at the feto-maternal interface as well as at the systemic level [1], [2], [3]. Such immunological changes may suggest a natural improvement of primarily Th1-mediated vasculitis (mainly Takayasu arteritis and Behçet disease which are the most prevalent vasculitis during pregnancy) and a worsening of Th2-driven ones, such as Wegener granulomatosis or Churg–Strauss syndrome. The course of the different vasculitis in pregnancy appears to be affected by several factors and whether or not such changes are able to modify maternal and fetal outcomes in each specific vasculitis is still uncertain.
These conditions should be managed very carefully during pregnancy, since both disease complications and pharmacological treatment may have negative effects on maternal and fetal health. Furthermore, treatments mainly based on the use of cytotoxic and immunosuppressive drugs may have a detrimental effect on the fetus.
To date, variations in disease activity of the different vasculitis during pregnancy and their relationship with the modification of immunoendocrine environment have not been evaluated. Most of the studies have mainly investigated the effects of disease complications on maternal and fetal outcomes, thereby including organ failure or irreversible vascular lesions.
This systematic review is focused on the relationship between pregnancy and systemic primary vasculitis. Guidelines for the management of these conditions during pregnancy are also suggested.
Section snippets
Search strategy
We performed a systematic review of the literature to find out all the cases of pregnancy in women affected with different vasculitis reported from 1960 to 2011, using the PubMed, Cochrane and Embase databases. Moreover, we hand-searched for the relevant articles referenced in other publications and not available on the web database.
Every report in English and in English-written abstracts concerning pregnant women affected with the following vasculitis were included in the analysis: Takayasu's
Search results
As summarized in Fig. 1, the initial search, based on the search terms, led to the finding of 2319 titles. Among these, 242 titles were pertaining to the search. By reading the abstracts, 82 of them were excluded because they did not meet the inclusion criteria and 160 were selected. Furthermore, 8 case reports were excluded after discussion by the two blinded Authors because they did not find a full agreement on the description accuracy of the cases and two were excluded because they had been
Takayasu's arteritis
TA is a granulomatous vasculitis which affects large vessels such as aorta, its major branches, and the pulmonary arteries. TA typically occurs in women during their childbearing age, therefore it is more common to observe pregnancy in patients with TA than in those with other vasculitis [4].
We have been able to identify TA in the literature 214 pregnancies in 168 patients affected with [5], [6], [7], [8], [9], [10], [11], [12], [13], [14], [15], [16], [17], [18], [19], [20], [21], [22], [23],
Polyarteritis nodosa
PAN is a disorder characterized by necrotizing inflammation of medium size or small arteries. In patients with PAN prevalent features are general symptoms, musculoskeletal, skin and gastrointestinal manifestations, and peripheral neuropathy, especially mononeuritis multiplex [4].
Nineteen case reports on pregnancy in PAN patients are available in the literature (Table 2) [52], [53], [54], [55], [56], [57], [58], [59], [60], [61], [62].
Wegener's granulomatosis
WG is an uncommon, small-vessel, necrotizing vasculitis which usually affects upper respiratory tract, lungs, and kidney. The disease peaks after the age of 40, thus, pregnancies in women with WG are uncommonly observed [4]. To date 48 pregnancies in 38 patients are available in literature [62], [63], [64], [65], [66], [67], [68], [69], [70], [71], [72], [73], [74], [75], [76], [77], [78], [79], [80], [81], [82], [83], [84], [85], [86], [87], [88], [89], [90], [91], [92]. Disease started during
Behçet's disease
BD is a chronic, relapsing, multisystemic, inflammatory process characterized by recurrent oral and genital ulcers, ocular, gastrointestinal, neurological manifestations, and thrombosis. It predominantly affects young women during childbearing age, therefore it is not rare to observe pregnancy in patients with BD [4].
Data regarding the reciprocal influence of BD and pregnancy derive from the analysis of 229 pregnancies in 131 patients diagnosed with BD, obtained from several case reports, five
Other vasculitis
So far, we have discussed the most common vasculitis that may affect gestational outcome. Although less frequently, other vasculitis have been reported in pregnant women.
Conclusions
The modulation of immune functions induced by pregnancy period may influence the course of vasculitis, which may in turn affect fetal or maternal outcome [4].
Unfortunately, no extensive data on pregnancy in patients with systemic vasculitis are available, due to the low incidence of such diseases, the low female-to-male ratio and disease onset, which often occurs after childbearing age. Most of the information we have derives from case reports and retrospective studies, since long term
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