INTRODUCTION:Granulomatosis with polyangiitis (GPA), formerly called Wegener's granulomatosis, is a rare small-medium vessel necrotizing vasculitis which usually presents as a systemic disease with sino-nasal, pulmonary, renal, and cutaneous manifestations. It has rarely been described in pregnancy and is a challenge to diagnose and treat due to the paucity of relevant literature. In addition, only a handful of cases have reported a de-novo diagnosis during pregnancy, usually in the second and third trimesters.[1] The constellation of features seen in our patient, namely respiratory failure requiring ICU admission and GPA diagnosis in the first trimester a twin pregnancy make it a unique presentation.CASE PRESENTATION: Our patient is a 22-year-old female in her second pregnancy who presented to the emergency room (ER)at 12 weeks of gestation with acute onset fevers, cough, vomiting, and night sweats. She had a history of recurrent epistaxis, sinusitis, and subglottic stenosis, recurrent pneumonia, and lung abscesses with bilateral pulmonary cavitary lesions on CT. She had undergone flexible bronchoscopy where biopsies showed granulomatous inflammation. Upon arrival to the ER, she was tachycardic, tachypneic, and febrile, requiring 15 liters of supplemental oxygen. Labs showed leukocytosis and elevated procalcitonin. Chest X-ray revealed worsening bilateral infiltrates with a left-sided pleural effusion. Urinalysis showed elevated protein and red blood cells. ESR 137, C-reactive protein 33.8 c-ANCA was 1:80; anti-proteinase 3 antibody-positive, myeloperoxidase antibody negative, IGG4 -77, collagen Type II Ab: 8.6 Respiratory cultures were positive for Serratia and pseudomonas. Fungal and anaerobic cultures were negative. She was initiated on broad-spectrum IV antibiotics but had no improvement. Due to high clinical suspicion for GPA, she was started on IV methylprednisolone 60mg BID as well as amphotericin for fungal coverage. Within 24 hours of starting steroids, significant improvement was noted which prompted dose escalation to 1000mg daily for 3 days. The patient continued to improve and was discharged home after 12 days of hospitalization with oral steroids and plans to start azathioprine outpatient DISCUSSION: Immunosuppressive therapy is the cornerstone for the treatment of multisystem Wegner's disease. However, treatment is challenging in the first trimester due to fetal adverse events reported with commonly used drugs like methotrexate, cyclophosphamide, and rituximab. A combination of glucocorticoids and azathioprine is safe and effective in pregnancy with IVIG being reserved for refractory cases. [2,3] CONCLUSIONS: Our case adds to the limited repository of cases with de novo-GPA in pregnancy and shows safe and effective treatment with high-dose steroids.
