Chest Pain of Atypical Cause in a Young Man

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  Figure 1. A 25-year-old man was admitted to the National Tuberculosis and Lung Diseases Research Institute in January 2020 for recurring chest pain. In November 2016, granulomatosis with polyangiitis (GPA) involving the ear, nose, throat, lungs, kidneys, and skin was recognized, with the presence of antiproteinase 3 (PR3) antibodies. He was treated with standard immunosuppressive agents (oral cyclophosphamide, 2 mg/kg; and prednisone, 0.8 mg/kg) [1] and achieved complete remission in November 2018, but experienced recurrent chest pain in March 2019. The pain lasted for 4–6 days and recurred every 3–4 months, without shortness of breath, fever, or other symptoms. On admission, the patient was in good condition. His physical examination results were unremarkable, without respiratory or cardiac pathological findings upon auscultation. A complete blood count showed leukocytosis (14,000 cells/μL) with neutrophilia (10,000 cells/μL). Serum biochemistry revealed an elevated C-reactive protein level of 234 mg/ml (CRP; normal range, <5.0 mg/mL) and increased PR3 antibody levels compared with the most recent results from November 2018 (28 >75 U/mL). There was no renal dysfunction or changes in urine…

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  Figure 2. To the best of our knowledge, GPA with the only recurrent symptom being chest pain caused by cardiac involvement has not been reported. In general, clinical signs of cardiac involvement in GPA are rare and commonly associated with coronary artery or pericardial involvement [4]. Echocardiography is usually a safe and easily accessible method, but its sensitivity and specificity are limited [5]. CMR is most accurate in diagnosing cardiac lesions in vasculitis [6] and is reliable for monitoring treatment efficacy [7]. Nonetheless, specific abnormalities in CMR associated with GPA remain undefined. Therefore, despite its invasive nature and limited sensitivity, EMB remains the gold standard for diagnosing myocarditis [8]. In our patient, this procedure was essential for the final diagnosis. Histological documentation of cardiac involvement in GPA is uncommon. In a series of GPA cases, unequivocal cardiac involvement was found in less than 2% of cases [3]. The low diagnostic accuracy of EMB is likely due to the rate of sampling errors [8]. Additionally, the patchy cardiac lesions complicate diagnosis [9]; therefore, both experience and close cooperation between clinicians and…

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  Figure 3. Clinically overt cardiac involvement in GPA is rare (3.3%) [4]; therefore, it may be easily overlooked. Pericarditis is the most common symptom in symptomatic patients (35%) [4], but only a few reports have described myocarditis caused by GPA [10]. Although pericarditis is usually mild, it can relapse or progress to constrictive pericarditis and tamponade [11], whereas myocarditis can lead to congestive heart failure or life-threatening arrhythmia [12]. Cardiac involvement in GPA increases mortality and the risk of relapse [13,14]. Therefore, the possibility of this condition should be considered, particularly when chest symptoms are present. However, cardiac involvement may be clinically silent [15]; therefore, CMR monitoring should be recommended in all GPA patients, irrespective of symptoms or abnormalities on basic cardiac tests. In conclusion, we present a rare case of thoracic pain caused by cardiac involvement in recurrent GPA, without any other symptoms. The diagnosis was made by combining several complementary imaging modalities, including cardiac histology. This case demonstrates the great challenge in diagnosing GPA, particularly in cases with single-organ involvement.…