Volume 16 (2019): Issue 6 (December 2019)

Aim. The prevalence ratio of systemic lupus erythematosus in men compared to women is 1:10, with more aggressive forms in men and in children. The incidence of lupus nephritis is of 50-60% in patients with lupus.

In this paper, we aim to report on a series of cases that include male patients who had lupus nephritis via renal biopsy and were admitted between January 2011 - December 2017, with or without other SLE manifestations. The aim is to review the particularities and the therapeutic response: age at onset, disease duration, lupus nephritis class, extra-renal organ involvement of lupus disease, paraclinical findings – proteinuria, inflammatory syndrome, renal function, therapeutic response – immunosuppressive regimens used for induction and maintenance, remission onset, remission type, number of flares, side effects of immunosuppressive therapy.

Materials and method. We have reviewed the histopathology database of male patients with lupus nephritis revealed via renal biopsy, medical charts and the Hippocrates information system in order to collect patient data.

Outcomes. Out of 68 renal biopsies positive for lupus nephritis, 9 were from male patients, which reveals a 6.5:1 ratio. The average age at the time of the renal biopsy is 37. 33 years old. Lupus nephritis class - 8 out of 9 patients were class IV, 1 sample was class IV+V. The duration of the corticosteroid therapy is 6.6 years. In addition, we also reviewed the immunosuppressive agents used, the number of flares, and the side effects of the medication.

Conclusions. Our data are similar to the literature data.

Objectives. Our study aimed to compare to what extent the characteristics of patients with ankylosing spondylitis (AS) differ, depending on the presence / absence of peripheral manifestations of the disease.

Materials and method. We analyzed a batch of 124 patients admitted to the rheumatology clinics of two hospitals, with ankylosing spondylitis according to the modified New York criteria. All individual patient characteristics were documented from patient observation sheets. We divided the population into two groups: the P + group representing the patients with peripheral manifestations of arthritis or enthesitis (N = 56) and the P- group representing the patients without the peripheral manifestations (N = 68). Statistical processing was performed using IBM SPSS and Microsoft Excel.

Results. In the subtype of patients P+ the mean age was 45.14 14.16 years, and in the subtype P- mean age was 43.84 years 12.58 years. Gender distribution of patients in the two subgroups shows higher frequencies for patients without peripheral manifestations P- in both genders, respectively 55.4% in males and 52.2% in females. The highest frequencies were registered at the normal BMI of 18.5-24.9 in both sublots, respectively 51.8% for the P+ patients and 45.6% for the P- patients. The degree of activity of the disease, based on the ASDAS - CRP score, within both P + and P- subgroups, revealed a majority of patients with inactive disease (≤ 1.3). Within the P+ and P- sublots, most patients had the BASDAI index in the range 0-0.9, that is 67.9% of the patients in the P+ sublot and 64.7% of those in the P- sublot, respectively. This shows that the majority of patients in both subgroups had a good therapeutic response to biological treatment, with symptoms such as pain and / or fatigue absent or very low in intensity.

There were no statistically significant differences between the two subgroups.

Conclusion. Even though there were no major paraclinical differences between the patients with pure axial ankylosing spondylitis and patients with AS with peripheral manifestations, the patients with peripheral manifestations of the disease should be approached by a multidisciplinary team.

Systemic lupus erythematosus (SLE) is considered the prototype of autoimmune diseases, the most complex autoimmune pathology and it is characterized by a wide range of immune processes, important antibodies production as well as an impressive spectrum of clinical manifestations. The great variety of lupus signs and symptoms caused difficulties in establishing well-defined classification criteria, as well as sustaining the clinical diagnosis.

In 2019, a joint initiative of European League Against Rheumatism (EULAR) and American College of Rheumatology (ACR) released a new set of classification criteria for SLE, worldwide SLE experts were involved, this being the largest SLE classification effort up to date.

Abdominal contrast enhanced ultrasound (CEUS) is a relatively new investigation method that combines conventional ultrasound with a small amount of intravenous contrast through a peripheral vein (usually the cubital vein). The contrast is made by very small gas microbubbles floating in a phospholipid suspension. The size of microbubbles are between 1-10 micrometers, being to large to go out of the vessels, but sufficiently small to stay into the capillaries, and this is the reason for they can be used as vascular tracers. So, it is possible to obtain images with microvessels inside of different organs in different vascular phases (arterial, portal, venous), images that are similar with the one obtained in CECT and CEMRI.

The intravenous contrast used in CEUS eliminates trough the lungs, during expiration. These is the reason why the most important contraindication for using iv contrast is severe respiratory failure. Also, it is important that intravenous contrast is not allergenic, the percentage of allergic reaction being extremly small (0,001%).

The most important advantages of CEUS are:

• It can be safely used for the pacients that are allergic to intravenous contrast used in CECT and CEMRI;

• It can be used in pacients with chronic kidney disease;

Abdominal contrast enhanced ultrasound is used in day-to-day practice mainly for evaluation of hepatic lesions, but also for pancreatic, kidney, testis pathology, as well as for inflammatory bowel diseases.

Advanced heart failure (AHF) is the stage of heart failure (HF) refractory to maximal medical treatment, cardiac devices (CRT - cardiac resynchronization, ICD - implantable defibrillator) and surgical treatment. AHF has become of interest through the experience and favorable results of treatment by mechanical circulatory support (MCS) and cardiac transplant (CT). The article reviews the criteria for defining the AHF (2018 ESC statement), natural history and prognosis outside the advanced treatment forms. Evaluation of risk and prognostic factors is required before the decision of advanced therapy: clinical factors (HF severity and reduction of cardiorespiratory functional capacity), RV function, biological markers and elements of organ dysfunction, as well as reduction of tolerance to conventional medication.

Finally, the principles of treatment and the results of mechanical circulatory support and cardiac transplant are presented.

Introduction. Ischemic heart disease is the leading cause of death with increasing prevalence. Acute myocardial infarction is a consequence of prolonged acute myocardial ischemia, which appears secondary to an imbalance between oxygen consumption and intake at this level.

Case report. We present the case of a male patient, aged 53 years old, admitted in the Cardiovascular Recovery Clinic for moderate dyspnea, intermittent dizziness and muscle pain predominantly in the lower limbs. Regarding his medical history, he suffered an antero-lateral myocardial infarction due to excessive physical effort, which was trombolysed. Following the remission of the acute episode, the evolution over the next 6-12 months was towards heart failure clinically manifested by dyspnea. Considering the presence of heart failure associated with severely diminished ejection fraction, the medication is adjusted and Sacubitril/Valsartan is introduced at a dose of 49/51 mg twice a day, under which our patient presents with both clinical and echocardiography improvement. Cardio-pulmonary stress testing is the most accurate as it provides the best information regarding functional capabilities, beyond the ejection fraction of the left ventricle. Following the treatment with RNAi (angiotensin receptor-neprilysin inhibitor), the parameters evaluated during the stress test were improved, which is clinically transposed by improving the quality of life and implicitly the long-term prognosis.

Conclusion. The particularity of this case consists in the occurrence of myocardial infarction at a young age (36 years) in a patient without a heredocolateral history or associated risk factors at that time. The association of ARNI (Sacubitril / Valsartan) in the therapeutic scheme has determined a clinical improvement, as well as paraclinical especially regarding the echocardiographic parameters (the ejection fraction increased from 25% to 40% at the end of the evaluation).

A 38-year-old man was admitted to our department with moderate exertional dyspnea, fatigue and a syncope during exercise. The medical history revealed: left pulmonary sarcoma at the age of four, treated with radiotherapy, chemotherapy and left total pneumonectomy. At admission, laboratory tests showed high BNP (1426 pg/ml), normal calcium and parathormon levels. The transthoracic echocardiography found normal left ventricular (LV) systolic function with severe calcifications of the papillary muscles, mitral annulus, apical segments of the inferior septum and inferior wall (panel A - arrows; panel D) associated with severe mitral regurgitation (panel B). It also revealed severe tricuspid valve regurgitation, severe pulmonary hypertension (estimated to 120 mmHg, panel C) and small amount of pericardial fluid. A thoracic computed tomography described severe cardiac calcifications (panels E and F, arrow), a hypertrophic right lung herniated in left hemythorax and no other pathological findings in the remnant lung tissue. The pneumological evaluation noted a severe restrictive dysfunction. In this case, pulmonary hypertension was most probably determined by the left cardiac disorders (severe mitral regurgitation postradiotherapy, LV diastolic dysfunction due to severe myocardial and papillary muscle calcifications), most probably related to thoracic radiotherapy during childhood.

A 43 year old male was referred to our center for assessment of the cardiac involvement in Erdheim-Chester disease (EHD) by cardiac magnetic resonance (CMR). The patient presented with a history of bone involvement as well as retroperitoneal mass, demonstrated to consist fibrosis as well as histiocyte infiltration.

The CMR examination included cine (SSFP – steady state free procession), T1 weighted (T1w) and T2 weighted (T2w) sequences, as well as late enhancement images 10 minutes after gadolinium based contrast injection (0.2 mmol/kg). The acquired images showed normal dimensions and function for both right and left cardiac chambers. However, a cardiac mass was revealed in the free wall of the right atrium and the junction with the right ventricle, with clear borders and a diameter of 2.5 cm (Image 1). The tissue was best viewed on axial sequences and was isointense on cine, T1w and T2w images and was mildly enhanced on LGE images. Consequently, the diagnosis of cardiac involvement in EHD was confirmed. The patient was started on specific treatment for EHD and 3-year CMR follow-up showed regression of cardiac involvement.

Erdheim-Chester disease is a rare disorder most frequently characterized by non-Langerhans histiocytic multifocal osteosclerotic lesions, with multisystemic granulomatosis and widespread manifestations, as well as highly variable severity⁽¹⁾. ECD affects the cardiovascular system in 75% of patients with infiltration of the pericardium and the right atrioventricular septum being the most common presentation. Typically the mass appears isointense on T1 and T2 weighted images and has low contrast enhancement, as was the case in our patient. In approximately 60% of cases death occurs because of cardiac complications, like pericardial tamponade, myocardial infarction, cardiomyopathy or arrhythmias⁽²⁾. Patients may sometimes be successfully treated with biologic therapy, interferon alpha or radiotherapy.