ABSTRACT
Xanthogranulomatous pyelonephritis (XGP) and Plummer-Vinson Syndrome (PVS) are two rare disorders that pose considerable diagnostic difficulties mainly because their signs overlap and are multifaceted. XGP is a severe form of pyelonephritis imitating cancer, whereas PVS is defined by dysphagia, iron deficiency anemia, and esophageal webs. This article presents the case of a 53-year-old female with a previous history of renal calculi and multiple transfusions who presented with dysphagia, flank pain, and hematuria. Findings from investigations showed severe anemia, a renal lesion suggesting malignancy, and GI findings pointing to the presence of PVS. The coexistence of XGP and PVS in this patient highlights the need for careful differential diagnosis and the importance of a multidisciplinary approach in managing patients with rare overlapping syndromes. Furthermore, this example shows how chronic infection, malnutrition, and their potential for leading to neoplasms connect. In summary, one should recognize that the possibility these two conditions can coincide is paramount for accurate diagnosis and effective management. Lastly, this case demonstrates that comprehensive assessment can only be achieved through coordinated care addressing both direct effects as well as secondary complications due to such uncommon diseases.
ABSTRACT
BACKGROUND: Acute chest pain is a common and challenging clinical presentation, necessitating rapid and accurate differentiation between potentially life-threatening etiologies like acute coronary syndrome (ACS) and acute myocarditis. The Salzburg Myocarditis Score (SMS), designed to aid in the early detection of myocarditis, offers a structured approach to this diagnostic challenge. However, the lack of a reliable clinical score for differentiating between these two conditions has been highlighted in recent literature, particularly in the context of limitations in using troponin levels alone for myocarditis diagnosis. OBJECTIVE: This study aimed to assess the diagnostic accuracy of the SMS for differentiating ACS and myocarditis in adult patients presenting with acute chest pain at Saveetha Medical College, Chennai, India. METHODS: A retrospective observational cohort study was conducted involving 100 consecutive patients presenting with acute chest pain. The SMS was calculated for each patient, and the final diagnoses of ACS or myocarditis were confirmed through comprehensive cardiac imaging (echocardiography or cardiac MRI) and additional biomarker analysis, following recommendations from established guidelines. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and a chi-square test were employed for statistical analysis. RESULTS: Among the 100 patients, 60 were diagnosed with ACS, and one was diagnosed with myocarditis. The SMS demonstrated high sensitivity (84.09%) and specificity (88.76%) for ACS, aligning with previous research findings. However, for myocarditis, the sensitivity was notably lower (25.81%), while specificity remained high (95.12%), consistent with concerns raised about the limitations of the score in identifying myocarditis. The PPV and NPV for ACS were 60% and 100%, respectively, while for myocarditis, the PPV and NPV were 2.5% and 100%, respectively. A chi-square test revealed a significant association between SMS predictions and the final diagnosis (p<0.001). CONCLUSION: The SMS is a valuable tool for identifying ACS in patients with acute chest pain. However, due to its low sensitivity for myocarditis, additional diagnostic tests, such as cardiac MRI, are crucial when myocarditis is suspected, despite a low SMS.
ABSTRACT
Bullous pemphigoid is an autoimmune blistering disorder predominantly affecting the elderly, with rare systemic complications, including cardiac involvement. This case study presents a 46-year-old female with a history of arterial hypertension who developed complete heart block (CHB) associated with bullous pemphigoid. The patient initially presented with bilateral lower limb swelling and discoloration, later diagnosed as bullous pemphigoid following the appearance of characteristic skin lesions. Concurrently, she was found to have asymptomatic bradycardia, with an electrocardiogram confirming CHB. After ruling out other causes of heart block, a dual-chamber pacemaker was implanted, and the patient was treated with immunosuppressive therapy to control the autoimmune blistering disorder. This case highlights the rare but significant association between bullous pemphigoid and CHB, emphasizing the importance of multidisciplinary care and timely intervention in managing such complex cases.