Search

Copyright of Studia Pneumologica et Phthiseologica is the property of TRIOS, spol. sr.o. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

The complement system consists of more than 50 soluble and membrane-bound proteins. It is a crucial part of the innate immune system. Individual components of the complement cascade are cleaved during the activation process into smaller fragments with specific biological functions. Activation of complement cascade is finished by formation of a membrane-attacking complex associated with lysis of the target cell. The main functions of the complement system include opsonization, chemotaxis, and clearance of immunocomplexes and apoptotic cells. Congenital disorders of the complement system are very rare diseases. The patients are prone to recurrent infections caused mainly by encapsulated microorganisms (pneumococcus, meningococcus, haemophilus and staphylococus) and most of them develop systemic autoimmune diseases (resembling systemic lupus erythematosus) during their lifetime. The secondary decrease of complement components may be caused by decreased liver production of complement components, excessive or prolonged activation of the immune system as a result of various immunopathological conditions or because of the formation of autoantibodies against the complement components. Basic therapeutic approaches include vaccination against meningococcus, pneumococcus and haemophilus and antibiotic prophylaxis. [ABSTRACT FROM AUTHOR]

Copyright of Studia Pneumologica et Phthiseologica is the property of TRIOS, spol. sr.o. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Secondary immunodeficiencies (SID) represent heterogeneous group of acquired impairment of immune systém function with diverse aetiology. It is mostly a combined disorder of both humoral and cellular components of the innate and adaptive immune responses. Except for various pathological states as diabetes mellitus, impairment of liver and kidney functions, protein-energy malnutrition or immunosenescence, also iatrogenic-mediated immune system impairments belong among the most important causes of SID. SID are associated with immunosuppressive or anti-inflammatory treatment used in malignant and autoimmune diseases [(immunosuppressant or disease-modifying drugs, nonsteroidal anti-inflammatory drugs, tyrosine kinase inhibitors or monoclonal antibodies against the CD20 molecule, tumour necrosis factor – (TNF-α) or IL-6 receptor], some other therapeutic procedures (splenectomy or radiotherapy) or present as an adverse events of treatment which at first glance do not interfere with the mechanisms of the immune system (neuroleptic and antiepileptic drugs, angiotensin-converting enzyme inhibitors or anticonvulsants). Mostly a mild impairment of immunoglobulin production or cellular function is induced, but sometimes clinically significant immunodeficiency develops (especially after treatment with anti-CD20 or anti-TNF-alpha monoclonal antibodies) that already require therapeutic intervention. Therefore, these adverse effects of the treatment should be considered. If patients suffer from clinical symptoms typical for significant immunodeficiency, immunological monitoring is appropriate. [ABSTRACT FROM AUTHOR]

Background: Idiopathic retroperitoneal fibrosis is characterized by the development of inflammatory infiltrates with marked fibrosis along the large retroperitoneal vessels. Rituximab in combination with glucocorticoids constitute an effective therapy, but the responses are not long-lasting. In other similar situations, addition of cyclophosphamide to the combination achieved longer and deeper responses. This was the reason to use the triple combination in this case., Case: A 56-year-old man came with four weeks lasting abdominal pain with CT finding of retroperitoneal fibrosis with unilateral ureteral occlusion. Biopsy confirmed retroperitoneal fibrosis with histological findings of IgG4-associated disease. Treatment with prednizone was poorly tolerated. Therefore, the patient was switched to the combination of rituximab 375 mg/m2 on day 1, cyclophosphamide 300 mg/m2 in infusion in days 1 and 15, plus dexamethasone 20 mg in infusion on days 1 and 15, repeated in a 28-day cycle., Results: Fluorodeoxyglucose (FDG) positron emission tomography (PET/CT) examination after 4 months of treatment showed a marked decrease in FDG accumulation and complete disappearance of the fibrotic mass. After 8 months, the induction therapy was followed by maintenance therapy with rituximab 1,000 mg plus dexamethasone 20 mg in 6-month intervals. Control PET/MR examination after 3 years is consistent with complete remission. The number of circulating plasmablasts correlated with the disease activity., Conclusion: Treatment of retroperitoneal fibrosis with the tripple combination of rituximab, cyclophosphamide and dexamethasone achieved a very rapid disappearance of pathological FDG accumulation and fibrotic retroperitoneal mass, with complete disappearance achieved after 4 months of treatment. After 3 years of maitenance therapy, the diesease is still in complete remission on PET/MR examination. We suggest to continue the maintenance therapy with rituximab because of some increase in the number of circulating plasmablasts after prolongation of the intervals between rituximab administration.

Immunosenescence is characterized by remodeling and dysregulation of immune system due to aging process. These changes affect innate as well as adaptive immunity. Due to the complexity of the physiological processes, in which the mechanisms of the immune system interfere, also other systems of the organism are affected by these changes. Thymus involution as well as chronic antigenic stimulation are the main causes of immunosenescence and lead to a proinflammatory setting of the organism. In addition to impaired immune response against infections or reactivation of latent infections, reduced response to vaccination or decreased antitumor immune surveillance, changes of the immune system in elderly are clinically reflected in the development of chronic diseases typical for older age groups, such as neurodegenerative or metabolic diseases. The mechanisms of immunosenescence can be at least partially influenced by an active lifestyle and adequate dietary measures.

IgG4 related disease (IgG4-RD) is a rare and relatively new group of systemic inflammatory diseases characterized by inflammatory, fibrotic or sclerotic involvement of one or more organs accompanied by increased IgG4plasma cells tissue infiltration andusually elevated serum IgG4(IgG4 > 1.35g/l, normal range 0.08-1.40 g/l) level. Histopathological findings are crucial for the diagnostics of this disease. The authors present a case report of a patient with IgG4 associated disease manifested by a rare combination of autoimmune hemolytic anemia and pulmonary involvement.

Secondary immunodeficiencies (SID) represents heterogeneous group of acquired impairment of immune system function with diverse etiology. It is mostly a combined disorder of both humoral and cellular component of the innate and adaptive immune response. SID occurs mainly in adulthood. Among the most important causes of SID development belong diabetes mellitus, impairment of liver and kidney functions, protein-energy malnutrition, splenic defects and immunosenescence. These deficiencies of immunity are clinically manifested by an increased frequency or unusual complications of common infections and occasionally by the occurrence of opportunistic infections. Diagnostic procedure is based on blood count and differential leukocyte count, biochemical examination, including determination of total protein and albumin concentrations, and biochemical urine analysis to eliminate protein losses. If clinically significant secondary defect of the immune system is suspected, immunological examination is indicated. Patients with clinical symptoms of SID can be treated with prophylactic antibiotic therapy, vaccination, substitution immunoglobulin therapy, or by immune system modifiers.

Immunoglobulin products contain specific antibodies in IgG class which are directed against wide range of pathogens. These products are obtained from plasma of healthy donors. Immunoglobulin derivates can be administrated by intravenous or subcutaneous route in substitution or immunomodulation indications. The administration can be accompanied by a risk of adverse events. The most common are pyretic reactions, chills, headache, nausea, malaise and muscle pain, nevertheless also severe systemic reactions can occur. Almost one third of the reactions appear during the first administration. The number and severity of reactions depends on administered dose, speed and the application way. Pre-existing comorbidities of the patients and presence of acute infection are also an important risk factors for adverse reactions. Therapy of side effects is mostly symptomatic.

Interstitial lung processes (IPP), or diffuse parenchymal lung diseases, are a broad group of diseases characterized by varying degrees of pulmonary fibrosis and inflammation affecting predominantly, but not exclusively, pulmonary interstitium. IPP mostly occur in adulthood with maximum manifestation between 40 and 70 years of age. Although IPP mostly present as a primary diagnosis, they also belong to the portfolio of pulmonary disorders in patients with primary immunodeficiencies. The authors present case reports of patients with interstitial lung involvement and primary immunodeficiencies [particularly those manifesting also in adulthood, such as common variable immunodeficiency (CVID), and cytotoxic T-lymphocyte-associated antigen 4 (CTLA-4) deficiency]. In addition, we report the case of silico-sis patient with severe lymphopenia. Therefore, in patients with newly diagnosed interstitial lung disease, congenital immune system disorder should be considered. Basic immunological laboratory examination of humoral and cellular immunity should be an essential part of the differential diagnosis algorithm for interstitial lung disease.

Common variable immunodeficiency disorders (CVIDs) represent a group of primary immunodeficiency diseases characterized by hypogammaglobulinemia and dysfunctional immune response to invading pathogens. Previous studies have indicated that CVID is associated with microbial translocation and systemic myeloid cell activation. The goal of this study was to determine whether patients with CVID display elevated systemic levels of markers of granulocyte activation and whether the levels are further influenced by intravenous immunoglobulin (IVIg) infusions. The plasma levels of granulocyte activation markers elastase and myeloperoxidase were determined using enzyme-linked immunosorbent assay (ELISA) in 46 CVID patients and 44 healthy controls. All CVID patients were in a stable state with no apparent acute infection. In addition, granulocyte activation markers' plasma levels in 24 CVID patients were determined prior to and 1 h following IVIg administration. Neutrophil elastase and myeloperoxidase plasma levels were significantly higher in CVID patients than in healthy controls. Systemic elastase levels were further increased following IVIg administration. In vitro stimulation of 13 CVID patients' whole blood using IVIg in a therapeutically relevant dose for 2 h resulted in a significant increase in plasma elastase levels compared to unstimulated blood. The data presented here indicate that CVID is associated with chronic granulocytic activation which is further exacerbated by administering IVIg. Increased myeloperoxidase and elastase levels may contribute to associated comorbidities in CVID patients.

A disease associated with immunoglobulin IgG4 is a rare unit with very variable symptoms. We describe the course and treatment of the disease in a patient who presented with multiple lymphadenopathy and infiltrates in the area of the retroperitoneum and pelvis and signs of chronic sclerosing pancreatitis. The disease was clinically manifested by a significant loss of weight, but also by a loss of perception of taste and smell. The diagnosis was made based on a high amount of IgG4 expressing plasma cells in the sampled tissue and an increased concentration of immunoglobulins of type IgG and mainly subclass IG4. Rituximab in 475 mg/m2 dose was used in the treatment, the initial four doses of rituximab were administered at 14-day intervals, always with a one-off administration of a 40 mg dose of dexamethasone. According to FDG-PET/CT, only partial remission of the disease was reached after 4 applications of rituximab and dexamethasone. The patient recovered its sense of smell and taste. In another 4 cycles ritu-ximab was administered on day 1 of a 28-day cycle. On days 1 and 15 of the cycle dexamethasone at 40 mg and cyclophosphamide at 600 mg were administered by intravenous infusion. After the completion of 8 cycles of treatment based on rituximab and dexamethasone and with cyclophosphamide added in the second half of the treatment, the control FDG-PET/CT examination proved the complete remission. Before the treatment commencement the concentration of the subclass of immunoglobulin IgG4 was equal to 51.0 g/l, after the completion of the aforementioned treatment it dropped to 3.5 g/l. The patient tolerated the treatment without any adverse effects. Ritu-ximab, dexamethasone and cyclophosphamide induced the complete remission of this disease.Key words: IgG4-associated/releated disease - rituximab.

Background: The aim of the study was to validate a hypothesis about the presence of a concomitant peripheral nerves injury in patients suffering from neurodegenerative disorders. The study was focused on the correlation between motor involvement in Parkinson's disease and peripheral nerves injury., Methods and Results: 23 patients suffering from Parkinson's disease, which was diagnosed in terms of strict UK-PDBB criteria, were examined. There were 14 men (mean age: 57 years, mean age at the disease onset: 51 years, mean disease duration: 7 years, mean duration of dopaminergic treatment: 4 years) and 9 women (mean age: 67 years, mean age at the disease onset: 63 years, mean duration of disease: 4 years, mean duration of dopaminergic treatment: 1 year). Polyneuropathy was clinically present and confirmed using EMG examination in 10 patients (43.5 %), 5 men and 5 women., Conclusions: Our observation indicates that the neurodegenerative process can be involved not only in the degeneration of central nervous system, but also in the peripheral disorders in this population.

Objective: To explain the mechanism of the origin of unilateral retrohyaloid and vitreous hemorrhage in case of subdural haematom after a severe brain injury., Methods: A detail examination by help of fluorescein angiography and funduscopy revealed a fluorescein leakage site in lamina limitans interna into the vitreous., Results: Fluorescein angiography revealed a fluorescein leakage site at the margin of the optic disc. It was possible to find the source of hyperfluorescence that increased its intensity over a time after vitreous hemorrhage had been removed., Conclusion: The diagnosis of Terson syndrome was started on the base of eye examination. The damage to peripapillary tissues demonstrated by fluorescein leakage suggests that intracranial hypertension affects peripapillary structures through the intervaginal space of the optic nerve sheath.