DEAR EDITOR, We describe a case of a 27-year-old male who presented to the hospital with a 3-week history of epistaxis, cough, chest pain, haemoptysis and bilateral lower extremity swelling.His past medical history was significant for post-streptococcal glomerulonephritis diagnosed at age 7 years, for which he received glucocorticoids for 3 years.Despite treatment, he progressed to chronic kidney disease stage 5.Other past medical history was notable for non-crystal-proven gout and three or four episodes of shortness of breath, wheezing, dry cough and sinus congestion over the past 2 years, all glucocorticoid responsive, with a presumed diagnosis of asthma.The patient resided in Ohio, without recent travel or tuberculosis exposure.He had never smoked or used recreational drugs.Physical examination at presentation revealed stable vital signs.The patient appeared pale and had friction rubs and bilateral crackles on cardiac and lung auscultation, respectively.He was noted to have diffuse sinus tenderness and nasal crusting, without nasal deformity or septal perforation.There was no evidence of a purpuric rash.Initial laboratory evaluations were notable for normocytic anaemia, with a haemoglobin of 7.6 g/dl (baseline haemoglobin 12 g/dl), leucocytosis of 16.39 x 10 3 /ll (79% neutrophils, 10% eosinophils, with absolute eosinophil count 1740/ll), elevated creatinine at 22.44 mg/dl, blood urea nitrogen of 223 mg/ dl, high-sensitivity troponin at 264 ng/l and B-type natriuretic peptide at >70 000 pg/ml.ECG showed sinus tachycardia, without ST segment elevation or T wave inversion.Plain chest radiography showed diffuse airspace opacities in both lungs.CT of the chest without i.v.contrast revealed Key message• Eosinophilic granulomatosis with polyangiitis can rarely present with life-threatening cardiac tamponade and responds to CSs and rituximab.
Background Cyclophosphamide (CYC) has known cytotoxic effects on ovarian reserve and has been linked to premature ovarian failure (POF) in systemic lupus erythematosus (SLE). The concurrent use of gonadotropin-releasing hormone agonists (GnRHas) is postulated to preserve ovarian function by reducing the number of follicles exposed to CYC, but there is paucity of data to establish its efficacy. We conducted a meta-analysis to summarize the effect of concurrent GnRHa use in persevering ovarian function and pregnancy. Methods English language databases of PubMed, Embase, and Cochrane were searched to include studies published between 2000 and 2021. Studies in females with rheumatic diseases receiving concurrent GnRHa and CYC therapy to evaluate ovarian preservation as defined by amenorrhea, follicle stimulating hormone (FSH), anti-mullerian hormone (AMH), or estradiol levels or successful pregnancy were included. We used a fixed effect, exact, Mantel-Haenszel approach to estimate the overall odds ratio (OR) and associated 95% confidence intervals (95% CIs). Results Seven studies with 218 female patients were included. The ovarian function was preserved in 125/132 (94.6%) of women who received GnRHa concurrently with CYC compared to 50/86 (58%) of women who did not receive GnRHa (OR = 10.3, CI = 4.83–36.29). The OR for pregnancy with GnRHa use = 2.94 (CI = 1.04–9.89). Conclusion Our results based on limited published studies suggest that concurrent GnRHa use preserves ovarian function and increase odds of pregnancy. It can be considered for premenopausal SLE females receiving CYC. Long-term follow-up studies are needed to establish the efficacy and safety of GnRHa use for ovarian preservation.
Amyopathic dermatomyositis (AMD) is a subtype of dermatomyositis characterized by more prominent involvement of the skin rather than muscle and often positive for melanoma differentiation-associated gene 5 (MDA-5) antibodies. The most frequent pulmonary involvement in MDA-5 positive AMD is nonspecific interstitial pneumonia. However, rare cases of pulmonary alveolar proteinosis (PAP) have also been reported. Here, we present a case of a 28-year-old male who was recently diagnosed with AMD presenting with shortness of breath and dry cough was eventually diagnosed with secondary pulmonary alveolar proteinosis. This case underscores the multidisciplinary approach and diagnostic challenges associated with the diagnosis of PAP secondary to the rarity of the condition.
Brucellosis is a rare zoonotic infection with a low annual incidence in the United States. Infective endocarditis secondary to brucellosis involving native or prosthetic valves is contemplated to be an extremely rare entity. As Brucella can present with non-specific sign and symptoms, clinicians need to have a higher degree of suspicion of Brucella endocarditis in culture-negative endocarditis patients, particularly those who have a history of exposure to farm animals. Timely diagnosis with appropriate management using antibiotics can prevent valvular damage and restore the valve's structural integrity. In this case report, we present a case of culture-negative, serology-proven Brucella endocarditis of native mitral valve, with an initial presentation of stroke that was successfully treated with combination antibiotic therapy.
Dengue fever is an arboviral infection spread by the Aedes mosquito with a wide spectrum of presentations encompassing simple flu-like illness to hemorrhagic manifestations. Hemorrhagic complications range from simple petechiae and purpura to gastrointestinal bleeding, hematuria, and severe central nervous system (CNS) bleeds. Herein we present a case of a 38-year-old male with dengue fever along with its hemorrhagic manifestations. Additionally, we conducted an extensive review of the literature to elucidate pathophysiology, diagnosis, and management of hemorrhagic manifestations in dengue fever.
INTRODUCTION: Clinically Amyopathic Dermatomyositis (CADM) is a subtype of dermatomyositis (DM) characterized by the presence of cutaneous lesions, without clinical evidence of muscle weakness.The presence of autoantibodies against Melanoma Differentiation-Associated protein 5 (MDA5) in this group is highly associated with rapidly progressive interstitial lung disease (RP-ILD) and a worse prognosis.We present a case of anti-MDA5 associated RP-ILD to illustrate the importance of screening for MDA5 in patients with CADM and DM.CASE PRESENTATION: Our patient was a 45-year-old woman with a history of hypothyroidism, seronegative inflammatory polyarthritis treated with hydroxychloroquine, and notably, a history of biopsy-proven DM with a negative myositis-specific antibodies (MSA) panel (which did not include MDA5 antibodies).She had been successfully treated for DM, and maintained in remission with oral steroids.Four years following the diagnosis of DM, she presented with insidious and gradually progressive dyspnea for over a year.Physical examination was unremarkable.Pulmonary function testing showed moderate restrictive lung disease.Chest X-ray was notable for bibasilar interstitial changes.Computed tomography (CT) scan of the chest revealed nonspecific, bilateral ground-glass opacities in the middle and lower lung zones, with associated bibasilar bronchiectasis in the affected areas (Figure 1).Bronchoscopic evaluation, including transbronchial biopsy, was performed, which was negative for malignancy or infection.Open lung biopsy was obtained via robotic-assisted thoracoscopy from the affected lobes, with histopathology revealing interstitial fibrosis with multiple fibrotic foci without clear evidence of honeycombing.The pattern was suggestive of usual interstitial pneumonia (UIP).Repeat testing for MSA revealed an elevated anti-MDA5 antibody level at 22 SI.The patient was consequently diagnosed with RP-ILD in conjunction with CADM, prompting the initiation of oral prednisone and azathioprine.DISCUSSION: Anti-MDA5 antibodies recognize the RNA helicase encoded by MDA5, a protein that is involved in innate immune processes.The presence of anti-MDA5 antibodies in patients with CADM has been linked to the presence of RP-ILD, and portends a poor prognosis.The mechanism of this autoantibody production is not fully understood.One meta-analysis found that the presence of these antibodies was 77% sensitive and 86% specific for the identification of RP-ILD.Additionally, anti-MDA5 antibodies can be utilized to predict clinical outcomes and monitor disease activity in these patients.Due to its high mortality rate from respiratory failure, immediate therapy with high-intensity combination immunosuppression is essential once the diagnosis is made.CONCLUSIONS: This case highlights the importance of screening for anti-MD5 antibodies in patients with CADM to elucidate their risk of developing RP-ILD.
Burkitt lymphoma (BL), a highly aggressive B-cell non-Hodgkin lymphoma (NHL), usually presents in children and young adults with large extranodal masses involving jaw bones, gastrointestinal tract, and central nervous system. The three main subtypes of BL are endemic, sporadic, and immunodeficiency variant. Extranodal involvement is common in each variant of BL, although muscle tissue involvement is distinctly rare. Mode of spread may be hematogenous or via direct extension of the primary tumor. In this report, we present a case of a 41-year-old male who presented with a palpable mass in the buttock leading to foot drop as the initial manifestation of BL. An exhaustive review of the literature failed to discover any previous reports of BL occurring in this location.
Glomus tumors are usually benign tumors of the glomus cells with the immunocytochemical and structural features of smooth muscle cells. The majority of the cases of glomus tumors are benign but, rarely, they demonstrate malignant features both clinically and histologically (also known as glomangiosarcomas). Although glomangiosarcoma involving extracutaneous sites is uncommon, a few cases have been reported. A glomangiosarcoma of the heart is extremely rare due to the rarity of glomus bodies in the myocardium. In this case report, we present the case of a 31-year-old female with glomangiosarcoma involving the heart with an unknown primary lesion.
Cyclophosphamide (CYA), also known as cytophosphane, is a medication used as a chemotherapeutic agent and immune suppressor. Its common adverse effects include nausea, vomiting, diarrhea, bone marrow suppression, hemorrhagic cystitis, alopecia, lethargy, and cardiotoxicity. Cyclophosphamide-related cardiac toxicity is not uncommon and causes potentially serious complications in patients. In this review, we present a case of a 65-years-old patient who developed atrial fibrillation with rapid ventricular rate (RVR) after receiving a single dose of CYA. In this case, the advanced age of the patient, pre-treatment with prednisone, and renal insufficiency most likely predisposed the patient to CYA-induced cardiac toxicity. A relevant literature review was also conducted to determine the pathogenesis, risk factors, and spectrum of CYA-induced cardiac toxicity.