Systemic Lupus Erythematosus Presenting as Myopericarditis with Acute Heart Failure: A Case Report and Literature Review.

Acutely decompensated dilated cardiomyopathy in a middle-aged patient without the typical risk factor profile presents a clinical dilemma. While cardiomyopathy is a known aspect of systemic lupus erythematosus (SLE), initial clinical presentation as decompensated dilated cardiomyopathy (DCM) is exceedingly rare in the literature. We share the case of a 49-year-old African-American female with no past medical history who presented with overt heart failure of 4 weeks evolution. Workup showed acute onset decompensated dilated cardiomyopathy, with a serologic profile compatible with SLE. She responded well to immunosuppressive steroid therapy. Literature review for SLE presenting as dilated cardiomyopathy with acute heart failure revealed a paucity of clinical evidence and consensus. Therefore, a comprehensive review of case reports was undertaken. A total of 10 cases were identified. Patients were 90% female and averaged 31 years of age. Dyspnea was the most common clinical presentation, and dilated cardiomyopathy with severely compromised left ventricular function was universally appreciated. Clinical presentation to diagnosis averaged 2 weeks. Immunosuppressive therapy regimens were universally employed; however, the regimens varied significantly. High-dose steroid therapy was most commonly used, and clinical and functional recovery was reported in 90% of individual case reports. Within the limited evidence and experience of therapeutic approaches, the efficacy of different singular or combined therapy is based solely on anecdotal case reports. Given the near-complete response to a short course of high-dose steroid therapy as much in the clinical recovery as in the resolution of DCM, the limited evidence based on review of these observational case studies and series supports the initial use of high-dose steroid therapy in acute lupus myocarditis.

Chest Pain Due to Pericardial Effusion as Initial Presenting Feature of Rheumatoid Arthritis: Case Report and Review of the Literature.

Pericardial effusions are not uncommon in rheumatoid arthritis (RA); however, they are rarely the presenting symptom of the disease. We describe a 55-year-old female who presented to the emergency department with complaints of chest pain and dyspnea on exertion. Initial workup revealed a medium-sized pericardial effusion. The wide spectrum of etiologies, including infectious and non-infectious disease, was explored. Eventually, after ruling out an array of disease states, rheumatologic workup was positive for RA. The initial presentation in our case was atypical due to absence of small joint polyarthritis and other common symptoms of RA. In difficult cases, extensive workup including laboratory tests, electrocardiography, echocardiography and imaging studies can aid in narrowing the causes of pericardial effusion. This case demonstrates that pericardial effusion could be an early presenting feature of RA, even in the absence of more common symptoms, and should be considered in differential diagnosis.

Genetic susceptibility loci of idiopathic interstitial pneumonia do not represent risk for systemic sclerosis: a case control study in Caucasian patients.

BACKGROUND: Systemic sclerosis (SSc)-related interstitial lung disease (ILD) has phenotypic similarities to lung involvement in idiopathic interstitial pneumonia (IIP). We aimed to assess whether genetic susceptibility loci recently identified in the large IIP genome-wide association studies (GWASs) were also risk loci for SSc overall or severity of ILD in SSc. METHODS: A total of 2571 SSc patients and 4500 healthy controls were investigated from the US discovery GWAS and additional US replication cohorts. Thirteen IIP-related selected single nucleotide polymorphisms (SNPs) were genotyped and analyzed for their association with SSc. RESULTS: We found an association of SSc with the SNP rs6793295 in the LRRC34 gene (OR = 1.14, CI 95 % 1.03 to 1.25, p value = 0.009) and rs11191865 in the OBFC1 gene (OR = 1.09, CI 95 % 1.00 to 1.19, p value = 0.043) in the discovery cohort. Additionally, rs7934606 in MUC2 (OR = 1.24, CI 95 % 1.01 to 1.52, p value = 0.037) was associated with SSc-ILD defined by imaging. However, these associations failed to replicate in the validation cohort. Furthermore, SNPs rs2076295 in DSP (ß = -2.29, CI 95 % -3.85 to -0.74, p value = 0.004) rs17690703 in SPPL2C (ß = 2.04, CI 95 % 0.21 to 3.88, p value = 0.029) and rs1981997 in MAPT (ß = 2.26, CI 95 % 0.35 to 4.17, p value = 0.02) were associated with percent predicted forced vital capacity (FVC%) even after adjusting for the anti-topoisomerase (ATA)-positive subset. However, these associations also did not replicate in the validation cohort. CONCLUSIONS: Our results add new evidence that SSc and SSc-related ILD are genetically distinct from IIP, although they share phenotypic similarities.

Acute Myocardial Infarction Due to Coronary Artery Dissection in the Postpartum Period.

Background Though rare, myocardial infarction secondary to coronary artery dissection is a life-threatening event. In reproductive age women, it commonly occurs during pregnancy or the postpartum period. Case We present a case of pregnancy-related acute myocardial infarction due to spontaneous coronary artery dissection in a 37-year-old woman who presented to the emergency room with shortness of breath and sudden onset of retrosternal chest pain 8 days after delivery of premature twins. Coronary artery catheterization showed 75 to 90% stenosis in the left main coronary artery (LMCA), extending into the proximal and mid left anterior descending (LAD) branch. The LMCA appearance in the heart catheterization was consistent with vasospasm, but it was not responsive to medical management. Subsequently, she underwent a second coronary artery catheterization and was found to have dissection requiring emergent coronary artery bypass graft × 3 in the LMCA, circumflex, and LAD that was followed by an uneventful recovery. Conclusion Early diagnosis and management of myocardial infarction due to coronary artery dissection in the peripartum period is crucial. This condition should be suspected in young reproductive age women, even in the setting of minimal risk factors. Angiography is required for diagnosis. Management should be individualized as it may include both invasive and noninvasive measures.

A Rapidly Growing Abdominal Mass: Desmoid Tumor in Pregnancy.

Background Desmoid tumors are benign soft tissue tumors that locally invade adjacent tissue. There is a paucity of reports describing the rapid growth of these tumors during pregnancy. Case A giant desmoid tumor arising from the left abdominal wall of a young female patient with rapid growth during pregnancy is described. Preoperative evaluation included ultrasonography and magnetic resonance imaging. Decision made by a multidisciplinary team was not to intervene before birth, and abdominal delivery at term was accomplished. Conclusion Desmoid tumors should be part of the differential diagnosis in an abdominal wall tumor of rapid growth during pregnancy. Future studies are needed for better understanding of the pathogenesis, diagnosis, and treatment of desmoid tumors in pregnant women.