Serum levels of interleukin 17 and 22 in patients with systemic sclerosis: a single-center cross-sectional study.

Systemic sclerosis (SSc) is a chronic, autoimmune disease characterized by inflammation, vasculopathy, and fibrosis of the skin and internal organs. Immunological response in SSc is still poorly understood. Cytokines play a significant role in this process leading to vasculopathy and fibrosis. In this cross-sectional study, we aimed to investigate serum levels of Th-17-related cytokines, IL-17 and IL-22, and to determine their correlation to the clinical association in SSc patients. Serum IL-17 and IL-22 levels were examined in 42 SSc patients and 29 healthy individuals. Associations between serum IL-17 and IL-22 levels and the duration of the disease, the extent of skin fibrosis, capillaroscopic findings, and involvement of the internal organs were explored. Serum IL-17 levels were not different in SSc and the control group. Serum IL-22 levels were significantly elevated in SSc patients compared to healthy individuals (p = 0.04). A positive correlation was found between the IL-22 sera levels and interstitial lung disease (p = 0.007). These results suggest IL-22 as a potential biomarker in SSc-related interstitial lung disease.

Successful treatment with Cinryze® replacement therapy of a pregnant patient with hereditary angioedema: a case report.

BACKGROUND: Hereditary angioedema (HAE) is a rare disease characterized with recurrent swelling of subcutaneous or mucosal tissue that resolves in approximately 3 days. It can be presented with peripheral edema, abdominal and life-threatening laryngeal angioedema. A variety of triggers are known to cause episodes of angioedema including estrogen exposure. There are different reports regarding the effect of pregnancy on HAE attacks, and in some patients, the pregnancy is a recognized triggering factor. CASE PRESENTATION: We present a female Caucasian patient with pre-existing HAE and disease exacerbations during pregnancy, requiring prophylactic use of plasma-derived C1 inhibitor concentrate. She was treated with Cinryze® replacement therapy throughout the pregnancy 1000 IU i.v. 48 times. She gave birth to a healthy male infant, via C-section. After the delivery, the patient was symptom-free for 6 months and required no treatment for HAE. CONCLUSIONS: In the case presented, the angioedema attacks worsened as the pregnancy progressed. The treatment with Cinryze® replacement therapy was effective and safe during pregnancy, with no adverse effects on the infant.

Optic neuritis as a presenting feature of Behçet's disease: case-based review.

Behçet's disease (BD) is vasculitis affecting vessels of variable sizes characterized with recurrent oral and/or genital aphthous ulcers accompanied by cutaneous, ocular, articular, gastrointestinal, and/or central nervous system inflammatory lesions. The disease is characterized by recurrent attacks and remissions of different durations, which is one of the reasons why the diagnosis is, in most cases, made several years after the onset of first symptoms. We present a 24-year old male, with South Eastern European heritage, with relapsing bilateral optic neuritis as a first symptom of the disease, followed by aseptic meningitis 2 years, and vascular manifestation 3 years after onset, which finally led to the diagnosis of Behçet's disease. Vascular symptoms were thromboembolism of the right leg and aneurism of the right popliteal artery that due to the size required surgical treatment. The patient was treated with glucocorticoids, azathioprine and anti-tumor necrosis factor-alpha therapy, that proved to be the best treatment options for all manifestations of the disease. Based on our literature review, optic neuritis is a known and rare clinical feature of BD. To our knowledge, there are only several literature reports in which optic neuritis is the initial symptom of BD. Our case report and literature review emphasize the importance of recognizing optic neuritis without inflammatory eye disease as a possible presenting symptom of BD and accentuate detailed medical history review at each patient's visit.

[CLINICAL ENTITIES AND CHARACTERISTICS OF PAIN IN PATIENTS WITH RHEUMATIC DISEASES].

Musculoskeletal pain is the most common symptom present in almost all rheumatic diseases. Rheumatic diseases include more than 150 clinical entities. There is no uniform classification of rheumatic diseases. In general, we distinguish inflammatory rheumatic diseases, non-inflammatory degenerative articular diseases, systemic connective tissue diseases, metabolic disorders with articular manifestations, and regional and extended pain syndromes. According to the International Association for the Study of Pain (IASP), pain is defined as an unpleasant sensation associated with tissue damage or reported simultaneously with such damage. Pain has a physical, mental, and social component. In rheumatic diseases the pain is mostly chronic and may severely impair the patient's general condition. The defining criteria involve a period of more than 3 or 6 months, and according to some definitions more than 6 weeks. In most cases the pain is nociceptive rather than neuropathic. Musculoskeletal pain, especially chronic pain, is a global public health problem because of its prevalence, as well as the frequently associated muslculoskeletal function impairment and development of chronic pain syndrome, which can be considered as a separate clinical entity and requires a biopsychosocial treatment approach.

[CRYOGLOBULINEMICVASCULITIS AS A MANIFESTATION OF PARANEOPLASTIC SYNDROME--A CASE REPORT]. / KRIOGLOBULINEMICNI VASKULITIS KAO MANIFESTACIJA PARANEOPLASTICKOG SINDROMA--PRIKAZ BOLESNIKA.

Cryoglobulinemia refers to the presence of circulating cryoglobulins in the serum which may lead to organ damage and systemic response characterized by fatigue, arthralgias, purpura, glomerulonephritis and neuropathy. The disease mainly affects small and medium-sized blood vessels and causes vasculitis due to cryoglobulin-containing immune complexes. Mixed cryoglobulinemia (type I and II) are most often associated with infections, especially hepatitis C virus (HCV), but may occur as part of lymphoproliferative and autoimmune diseases. We present a 75-year-old male patient with acrocyanosis and digital necrosis of the left hand and purpura of the lower extremities. Since 2001 the patient was treated for Non-Hodgkin's lymphoma of the stomach (MALT) and since 2002 for Sjogren's syndrome. Extensive diagnostic procedures revealed cryoglobulinemia type II, but without evidence of HCV infection or relapse of lymphoproliferative disorder. Furthermore, poorly differentiated prostate adenocarcinoma was diagnosed and antiandrogen treatment was initiated. One year after the onset of symptoms acrocyanosis and digital necrosis, gastric adenocarcinoma was diagnosed, without metastatic disease, and surgical treatment was performed. However, postoperatively recurrence of new digital necrosis of the hands occurred along with clinical deterioration and multiple organ failure leading to lethal outcome. Association of cryoglobulinemia and solid tumors is rarely described in the literature, so it is very important to perform comprehensive diagnostic evaluation and detect potential underlying disease as soon as possible.

[Pathophysiology of osteoporosis]. / Patofiziologija osteoporoze.

The balance of degradation and bone formation is important for the normal process of remodeling and maintenance of bone mass. Genetic, hormonal, immunological and other factors affect bone remodeling throughout life. Disorder of bone turnover plays key role in the pathophysiology of osteoporosis and presents a complex process which involves bone cells, cytokines and their receptors. Nowadays, important role of RANK/RANKL/OPG system in the pathophysiology of osteoporosis is well known, but it is still important to investigate the intracellular signaling pathways in the development of osteoporosis and other metabolic bone diseases.