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.

Generalized Lymphadenopathy as Presenting Feature of Systemic Lupus Erythematosus: Case Report and Review of the Literature.

Lymphadenopathy could represent a vast spectrum of etiologies including infectious and non-infectious diseases. Besides proper history taking, physical examination, and laboratory investigations, a tissue diagnosis is often necessary to unmask the cause of generalized lymphadenopathy. Here we present a 23-year-old woman who was admitted for diffuse generalized lymphadenopathy, fatigue, malaise, weight loss, nausea, and bilateral lower extremity edema. She reported a history of seizures as well as stroke 2 years prior with no other medical conditions present. Although malignant and infectious etiologies were initially the primary targets for workup, her history of seizures and stroke remained a dilemma. Extensive workup for malignant and infectious diseases was unrevealing; however, rheumatologic workup was eventually positive for systemic lupus erythematosus (SLE). This case illustrates that extensive generalized diffuse lymphadenopathy may be a presenting feature of SLE and should be considered in the differential diagnosis of patients presenting with diffuse lymphadenopathy and constitutional symptoms.

Wegener's granulomatosis presenting as fever of unknown origin in an African-American male.

Wegener's Granulomatosis (WG) is a rare, Multisystem disease of the medium and small sized arteries and veins. It most commonly involves the upper respiratory tract, lungs, and kidneys and often presents as chronic fatigue, upper respiratory infection, sinusitis, and otitis media. Symptoms can include fever, weight loss and fatigue, though these are not usually the primary presenting complaints. development of the disease is highly skewed across ethnicities, with up to 98% of cases being reported in caucasians. We present the case of a 56-year-old African-American male who presented primarily with complaints of uncontrollable fever of unknown origin (FUO) for past two weeks with accompanying sore throat, nasal congestion, night sweats, malaise, and unexplained weight loss of 10 pounds over the past month. Treatment with antibiotics for one week prior to admission showed no relief of symptoms. Chest x-ray showed focal course markings in the right upper lobe. Urinalysis revealed microscopic hematuria and leukocyturia. Chest and abdominal CT scans revealed a right lower lobe pulmonary nodule and heterogeneous areas of enhancement in the spleen. Head CT revealed right mastoid opification. Labs revealed proteinase-3 antibody titer > 100, which is characteristic of WG. Steroids and cyclophosphamide were started with relief of presenting symptoms. Renal biopsy showed pauci-immune P-ANCA associated crescentic and focally necrotizing glomerulonephritis and vascilitis. This case is unique in that the patient presented with primary complaint of FUO. WG should be considered as a rule-out in cases of uncontrollable FUO, even if none of the classic triad of symptoms is present. Though rare, WG should be considered in cases involving non-Caucasian patients.