COVID-19- induced Granulomatosis with Polyangiitis:a Case Report and Literature Review
【Abstract】 Objective To report a case of Granulomatosis with Polyangiitis (GPA)with Diffuse alveolar hemorrhage(DAH)that developed following Coronavirus disease 2019 (COVID-19) in an adolescent boy, and perform a literature review to better understand this disorder.Methods Retrospectively analyze the data of a case of GPA following COVID-19 infection, to summarize the clinical characteristics of GPA following COVID-19 infection by searching the database (PubMed,Wanfang Data,CNKI) and comprehensively analyze the literature results. Results9 cases were reported,combined with our case,there were 10 cases of GPA following COVID-19 infection,including 4 males and 6 females,with an average age of(40.6±18.6)years,and the time interval between COVID-19 and diagnosis of GPA ranged from 1 day to 3 months for all 10 cases. The rate of mortality was 10.0%(1/10).Commonly seen clinical manifestations were cough(70.0%),dyspnea(50.0%),and Arthralgia/Myalgia(40.0%). CT scans showed ground-glass opacities,multifocal pulmonary nodules.Of all these patients, c-ANCA and PR3-antibody positivity were found .Conclusion COVID-19 pneumonia and COVID-19- induced new-onset GPA share many clinical and radiological features, making it challenging for clinicians to distinguish between the two. Our study also provided some clues about the diagnostic challenge of GPA induced by COVID-19.
【Key words】 Coronavirus disease 2019;granulomatosis with polyangiitis;Case report;Literature Review
Background Granulomatosis with Polyangiitis (GPA) is a potentially fatal multisystem disease marked by necrotising granulomatous vasculitis of the minor arteries and veins. This condition mostly affects the lungs, upper respiratory tract and kidney.Although the processes behind the development of GPA are unknown, several factors such as infection, medications, and environmental exposure may play a role in the disease’s pathophysiology by inducing autoimmunity[1, 2] .Since the advent of the Coronavirus disease 2019 (COVID-19)pandemic, several reports have described AAV(anca associated systemtc vasculitis) occurrence after COVID-19 , indicating the potential of COVID-19 to trigger the development of GPA .We herein present a case of new-onset GPA with DAH(diffuse alveolar hemorrhage) that developed following COVID-19 infection in an adolescent patient,this is the first case of GPA with DAH in an adolescent in Hunan province of China, which developed shortly after COVID-19 infection, and we review the literature of GPA following COVID-19 infection by searching the database,to summarize the clinical characteristics of GPA following COVID-19.
Case Presentation
A 16-year-old boy with no past medical history presented to our Hospital with a 1-month history of fever,cough and shortness of breath, accompanying symptoms were epistaxis and tinnitus.One month prior to the onset of illness, the patient was exposed to a COVID-19 positive sick contact, so at the onset of symptoms, our patient was tested and returned positive for COVID-19 nucleic acid test.Before coming to our hospital, the patient was given active anti-infective treatment in a local hospital, but the symptoms were still not controlled, and the O2 saturation(SPO2) gradually decreased. So he went to the superior hospital for further diagnosis and treatment.
On admission, he was fever (37.6 C) with tachycardia (105 bpm), blood pressure 97/60 mm Hg, respiratory rate 25 beats per minute, and SPO2 90% on ambient air. He had saddle nose and bleeded from the bulbar conjunctiva.Wet rales could be heard on auscultation of the lung.
Blood tests were remarkable for: leukocytosis (20.8X10^9/L,normal value 4-10.0X10^9/L) with neutrophils (17.48X10^9/L, normal value 1.5-8.0X10^9/L),thrombocytosis (700X10^9/L,normal value 100-300X10^9/L) . PCT (0.88ng/L, normal value0-0.05 ng/ml),ESR(112 mm/ h, normal value 0~15 mm/ h), C-reactive protein (80mg/dL, normal value 0-8 mg/L) and hypoalbuminemia with albumin 26.9g/L(normal value 35-55g/L) .Renal,liver functions and coagulation function were normal, and serum c-ANCAs ,PR3- antibody were positivity. COVID-19 nucleic acid test was negative.
On pulmonary CT showed multiple nodules and cavities in both lungs,creating“an island in the lung”(Fig. 1). Paranasal sinus CT scan showed bilateral maxillary sinus and left frontal sinusitis.No pathogenic bacteria were found in 2 rounds of alveolar lavage fluid mNGS (Metagenomic next-generation sequencing).
Pathological examination of the puncture specimen of the left lung mass showed that a small amount of lung tissue was examined, and fibrous tissue was significantly proliferated in some areas, with a large number of inflammatory cells infiltration.
The patients were identified as having GPA and met the 2022 classification criteria for GPA established by the American College of Rheumatology and European Association of Rheumatology[3]. He started receiving pulsed intravenous methylprednisolone(0.5 g/day for 3 days). Cyclophosphamide was not administered because of its negative effects, particularly on sperm function. Rituximab 500 mg was provided twice, spaced by two weeks, in place of cyclophosphamide.
During the course of treatment, the patient developed massive hemoptysis, and the oxygenation index further decreased.Diffuse hemorrhage in the airway was observed under bronchoscopy, and GPA combined with DAH was considered. After endotracheal intubation and plasma exchange, hemoptysis stopped and fever improved, but the patient eventually died from acute pulmonary embolism.