A Case of Granulomatosis with Polyangiitis in Pregnancy

By Rashid Naseem Khan¹, Aliya Naseem¹, Muhammad Athar Khan¹, Qurat ul Ain¹, Mamoona Hashim¹, Hafiza Laila Hashim Khan²

Doi: https://doi.org/10.36283/ziun-pjmd14-1/025

How to cite: Khan RN, Naseem A, Khan MA, Ain QU, Hashim M, Khan HLH A Case of Granulomatosis with Polyangiitis in Pregnancy. Pak J Med Dent. 2025 Jan ;14(1): 171-173. Doi: https://doi.org/10.36283/ziun-pjmd14-1/025.

Received: Wed, October 30, 2024 Accepted: Wed, December 11, 2024 Published: Fri, January 10, 2025.

Granulomatosis with polyangiitis, a rare autoimmune disease affecting organs such as the respiratory system, kidneys, and lungs, poses unique challenges in pregnancy. A 25-year-old woman with chronic liver disease, vasculitis, protein C and S deficiency, hypothyroidism, and gestational diabetes presented at 24 weeks gestation with shortness of breath, fever, and vomiting. Her thrombocytopenia was managed with transfusions, and regular prenatal ultrasounds were conducted. At 36 weeks, an elective cesarean delivered a healthy 2.4 kg baby. This case highlights the critical role of multidisciplinary care in achieving favorable outcomes in complex pregnancies affected by granulomatosis with polyangiitis.

Keywords: Granulomatosis    with     Polyangiitis, Wegener’ Granulomatosis, Granulomatous Vasculitis, ANCA-Associated Vasculitis, Pregnancy

Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis (WG), is a rare, chronic systemic disorder marked by granuloma formation and vasculitis^1,2. As an autoimmune condition, GPA causes inflammation in blood vessels, predominantly targeting the upper respiratory tract, lungs, and kidneys³. GPA is the most prevalent type of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), with proteinase 3(PR3) antibodies present in 85–95% of cases⁴. The antineutrophil cytoplasmic antibodies (ANCAs) are detected in most patients with active disease, the cause, however, remains unknown. Treatment involves immunosuppressive therapy, with cyclophosphamide (CYC) and corticosteroids⁵. The disease manifests in the fourth and fifth decades of life and is rarely linked to pregnancy. During pregnancy, it is a therapeutic challenge that calls for individualized care⁶.

A 25-year-old patient with G2P1+0 known case of NBNC chronic liver disease, ANCA +ve vasculitis (Wegener granulomatosis), protein C and S deficiency, hypothyroidism, and gestational diabetes mellitus presented on 24 weeks of gestation with complaint of SOB and fever for 1 month and vomiting for 3 to 4 days. Her labs showed thrombocytopenia for which she received 2 PCV transfusions.

During 1^st, 2^nd, and 3rd-trimester ultrasound scans were done. She felt quickening in 5^th month. On the 36^th week of gestation, she presented to the ER with a complaint of leaking. LSCS was planned due to a previous C-section and leaking. After all aseptic measures, the abdomen was opened by Pfannenstiel incision, rectus sheath cut, muscles were separated, the peritoneum was opened and the lower segment of the uterus was opened by transverse incision. A live baby weighing 2.4 kg was delivered by vertex presentation placenta and membranes were expelled out completely the uterus was closed back in layers and homeostasis was secured. Bilateral tubal ligation (BTL) was also done. Operative findings: liquor was scanty, adhesions were present and there was blood loss of about 800 ml, 4 platelets were transfused during the C-section. Treatment was done via prednisolone (5mg), azathioprine (200mg), and hydroxychloroquine (200mg).

LAB FINDINGS

Wegener’s granulomatosis is an autoimmune disorder characterized by granulomatous inflammation and necrotizing vasculitis, which can lead to significant morbidity and mortality if left untreated⁷. According to the available literature disease’s presentation during pregnancy is particularly challenging, as the treatment options are limited due to the potential effects on the fetus and the associated risks for both the mother and the developing child. Wegener’s granulomatosis that manifests during pregnancy may exhibit a more aggressive clinical course and may necessitate more intensive treatment compared to cases that arise at other times⁸.

One of the earliest reported cases of Wegener’s granulomatosis in pregnancy was published in 1983, where the disease appeared during the postpartum period and subsequently led to the interruption of a subsequent pregnancy⁹. The literature frequently highlights adverse pregnancy outcomes in women with active disease at the time of conception. The Vasculitis Clinical Research Consortium Patient Contact Registry found that pregnancy loss was more common in women who conceived after being diagnosed with systemic vasculitis, including AAV, compared to those who conceived before diagnosis (33.8% vs. 22.4%). Increased disease activity during pregnancy was also linked to a higher likelihood of preterm delivery. Additionally, a recent French study documented 19 pregnancies in 11 women with AAV, reporting elevated rates of complications¹⁰.However, there is geographic and ethnic variation in GPA epidemiology. Among pregnancies complicated by GPA, approximately 71% resulted in live births. However, adverse outcomes were noted, including fetal loss in 11.5% of cases and premature births in 17.2% of pregnancies¹¹. The management of GPA during pregnancy often involves immunosuppressive treatments such as cyclophosphamide and corticosteroids, which can complicate maternal health. One case highlighted a woman who experienced a flare of GPA at 34-35 weeks but ultimately delivered a healthy baby.⁶ Although there are no specific preventive measures, understanding risk factors can be crucial. GPA is more common in individuals of northern European descent and typically affects adults aged 40-65. Genetic predispositions and environmental factors may also play a role in the development of the disease¹².

Managing pregnancy in GPA patients demands preconception planning, precise clinical assessment, and aggressive treatment of active disease. Ideally, conception should be delayed for at least six months after achieving remission. A multidisciplinary team is essential for effective diagnosis and treatment.

Not applicable

The author(s) declared no potential conflicts of interest concerning the research, authorship, and/or publication of this article.

 The study was approved by the IRB, Liaquat College of Medicine & Dentistry.

No financial support for the research.

All authors equally contributed.

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