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1.
J Immunol Res ; 2024: 4283928, 2024.
Article in English | MEDLINE | ID: mdl-38699219

ABSTRACT

Objective: To characterize the eosinophilic granulomatosis with polyangiitis (EGPA) population from the POLVAS registry depending on ANCA status and diagnosis onset, including their comparison with the granulomatosis with polyangiitis (GPA) subset with elevated blood eosinophilia (min. 400/µl) (GPA HE) to develop a differentiating strategy. Methods: A retrospective analysis of the POLVAS registry. Results: The EGPA group comprised 111 patients. The ANCA-positive subset (n = 45 [40.54%]) did not differ from the ANCA-negative one in clinics. Nevertheless, cardiovascular manifestations were more common in ANCA-negative patients than in those with anti-myeloperoxidase (MPO) antibodies (46.97% vs. 26.92%, p = 0.045). Patients diagnosed before 2012 (n = 70 [63.06%]) were younger (median 41 vs. 49 years, p < 0.01), had higher blood eosinophilia at diagnosis (median 4,946 vs. 3,200/µl, p < 0.01), and more often ear/nose/throat (ENT) and cardiovascular involvement. GPA HE comprised 42 (13.00%) out of 323 GPA cases with reported blood eosinophil count. Both GPA subsets had a lower prevalence of respiratory, cardiovascular, and neurologic manifestations but more often renal and ocular involvement than EGPA. EGPA also had cutaneous and gastrointestinal signs more often than GPA with normal blood eosinophilia (GPA NE) but not GPA HE. The model differentiating EGPA from GPA HE, using ANCA status and clinical manifestations, had an AUC of 0.92, sensitivity of 96%, and specificity of 95%. Conclusion: Cardiovascular symptoms were more prevalent in the ANCA-negative subset than in the MPO-ANCA-positive one. Since EGPA and GPE HE share similarities in clinics, diagnostic misleading may result in an inappropriate therapeutic approach. Further studies are needed to optimize their differentiation and tailored therapy, including biologics.


Subject(s)
Antibodies, Antineutrophil Cytoplasmic , Eosinophilia , Registries , Humans , Male , Middle Aged , Female , Adult , Retrospective Studies , Eosinophilia/diagnosis , Eosinophilia/immunology , Eosinophilia/blood , Antibodies, Antineutrophil Cytoplasmic/blood , Antibodies, Antineutrophil Cytoplasmic/immunology , Granulomatosis with Polyangiitis/diagnosis , Granulomatosis with Polyangiitis/immunology , Aged , Churg-Strauss Syndrome/diagnosis , Churg-Strauss Syndrome/immunology , Churg-Strauss Syndrome/epidemiology , Peroxidase/immunology , Eosinophils/immunology
2.
Clin Exp Rheumatol ; 40(4): 720-726, 2022 May.
Article in English | MEDLINE | ID: mdl-35485410

ABSTRACT

OBJECTIVES: The study aimed to characterise the Polish population of (ANCA)-associated vasculitides (AAV) with respiratory involvement (RI), in comparison to the subgroup without lung manifestations and the other cohorts. METHODS: Retrospective analysis of the Polish population of AAV with RI was conducted, based on data from the POLVAS registry. Standard descriptive statistics, χ2 test, and Mann-Whitney U test were used to perform comparisons. RESULTS: Among 461 cases qualified to this study, there were 316 cases with RI (68.5%), 206 with granulomatosis with polyangiitis (GPA) (65.2%), 80 with eosinophilic granulomatosis with polyangiitis (EGPA) (25.3%) and 30 with microscopic polyangiitis (MPA) (9.5%). Proportion of RI in GPA, MPA, and EGPA accounted for 67.8%; 40.0%; 97.6%, respectively. The number of relapses was higher in the RI group (median 1.0 vs. 0.0; p=0.01). In the subgroup of combined GPA and MPA with RI, the trends toward higher proportion of deaths (11.7% vs. 5.7%; p=0.07), relapses requiring hospitalisation (52.2% vs. 42.4%, p=0.07) and relapses requiring admission to the intensive care unit (5.6% vs. 1.4%, p=0.09) were observed, median maximal concentration of CRP was higher (46 vs. 25 mg/l; p=0.01) and more aggressive treatment was administered. CONCLUSIONS: Prevalence of RI in the Polish population of AAV is similar to the values reported in the literature, however, the proportion observed in GPA is closer to those presented in Asian than Western European cohorts. RI seems to be associated with a more severe course of disease and its presence prompts more aggressive treatment.


Subject(s)
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis , Churg-Strauss Syndrome , Granulomatosis with Polyangiitis , Microscopic Polyangiitis , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/complications , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/drug therapy , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/epidemiology , Antibodies, Antineutrophil Cytoplasmic , Churg-Strauss Syndrome/complications , Churg-Strauss Syndrome/epidemiology , Granulomatosis with Polyangiitis/complications , Granulomatosis with Polyangiitis/drug therapy , Granulomatosis with Polyangiitis/epidemiology , Humans , Microscopic Polyangiitis/complications , Microscopic Polyangiitis/epidemiology , Recurrence , Registries , Retrospective Studies
3.
Pol Arch Intern Med ; 132(3)2022 03 30.
Article in English | MEDLINE | ID: mdl-34985235

ABSTRACT

INTRODUCTION: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is characterized by the presence of proteinase­3 (PR3) or myeloperoxidase (MPO) ANCA. In over 90% of cases, PR3­ANCA is associated with granulomatosis with polyangiitis (GPA). However, it is also rarely found in microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA). On the other hand, MPO­ANCA being characteristic of MPA (>90% of cases), is also found in about 40% of EGPA and 5% of GPA patients. On the ground of this overlap, clinical importance of ANCA specificity identification has been questioned. OBJECTIVES: In this study, we analyzed the clinical and demographic characteristics of AAV subgroups identified by ANCA serotype. PATIENTS AND METHODS: We conducted a multicenter study of AAV patients (417 GPA, 106 MPA, 102 EGPA; diagnosed between 1990 and 2016), included in the POLVAS registry. The data were systematically collected according to a standardized protocol. RESULTS: In the ANCA-positive group (anti­MPO, anti­PR3) a male-to-female ratio was 1:1, whereas in the ANCA-negative group it was 1:2, regardless of AAV diagnosis. Anti­MPO antibodies were present in significantly older patients. Patients with MPO+GPA and MPO+EGPA were older than those with corresponding ANCA­negative GPA and EGPA as well as PR3+AAV. Moreover, ANCA­negative AAV was characterized by a low risk of end­stage kidney disease and death. CONCLUSIONS: The presence and specificity of ANCA in AAV patients are related to sex and age, determine their organ involvement and influence mortality as previously shown. Patients with MPO­ANCA-positive AAV constitute a clinically homogeneous group, whereas PR3­ANCA-positive patients are much more clinically heterogeneous. ANCA-negative AAV patients are characterized by better prognosis. Thus, ANCA identification is an indispensable element and should not be omitted in establishing AAV diagnosis.


Subject(s)
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis , Churg-Strauss Syndrome , Granulomatosis with Polyangiitis , Microscopic Polyangiitis , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/complications , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/diagnosis , Antibodies, Antineutrophil Cytoplasmic , Churg-Strauss Syndrome/complications , Demography , Female , Granulomatosis with Polyangiitis/complications , Granulomatosis with Polyangiitis/diagnosis , Humans , Male , Microscopic Polyangiitis/complications , Myeloblastin
4.
Clin Exp Rheumatol ; 39 Suppl 129(2): 62-68, 2021.
Article in English | MEDLINE | ID: mdl-32896241

ABSTRACT

OBJECTIVES: ANCA-associated vasculitides (AAV) are a heterogeneous group of rare diseases with unknown aetiology and the clinical spectrum ranging from life-threatening systemic disease, through single organ involvement to minor isolated skin changes. Thus, there is an unmet need for phenotype identification, especially among patients with granulomatosis with polyangiitis (GPA). Patients with microscopic polyangiitis (MPA) seem to be clinically much more uniform. Recently, three subcategories of AAV have been proposed and described as non-severe AAV, severe PR3-AAV, and severe MPO-AAV. METHODS: In line with these attempts, we decided to use an unbiased approach offered by latent class analysis (LCA) to subcategorise GPA and MPA in a large cohort of Polish AAV patients included in a multicentre POLVAS registry. RESULTS: LCA of our AAV group identified a four-class model of AAV, including previously proposed three subphenotypes and revealing a fourth (previously not described) clinically relevant subphenotype. This new subphenotype includes only GPA patients, usually diagnosed at a younger age as compared to other groups, and characterised by multiorgan involvement, high relapse rate, relatively high risk of death, but no end-stage kidney disease. CONCLUSIONS: Based on multiple clinical and serological variables, LCA methodology identified 4-class model of AAV. This newly described fourth class of AAV may be of clinical relevance and may require prompt diagnosis and aggressive treatment due to the multiorgan involvement, high risk of relapse and marked mortality among these relatively young GPA subjects.


Subject(s)
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis , Granulomatosis with Polyangiitis , Microscopic Polyangiitis , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/diagnosis , Antibodies, Antineutrophil Cytoplasmic , Granulomatosis with Polyangiitis/diagnosis , Humans , Latent Class Analysis , Microscopic Polyangiitis/diagnosis , Peroxidase , Poland
5.
Reumatologia ; 58(5): 331-334, 2020.
Article in English | MEDLINE | ID: mdl-33227048

ABSTRACT

Nephrotic syndrome (NS) can be a symptom of many autoimmune, metabolic, or infectious diseases. Kidney involvement is often observed in the course of diabetes mellitus (DM) and systemic lupus erythematosus (SLE). The development of NS with coexisting SLE and DM generates serious diagnostic problems. In this paper, the authors present diagnostic and therapeutic dilemmas in a patient with long-lasting DM, SLE, and secondary antiphospholipid syndrome, in whom NS symptoms appeared. Histopathological examination of the kidney confirmed the diagnosis of lupus nephritis. Immunosuppressive and anticoagulant drugs were used. The authors demonstrated that the character of morphologic lesions in the kidney biopsy can help in diagnosis, nephropathy classification, and further therapeutic decisions, which are distinct in both diseases.

6.
Anaesthesiol Intensive Ther ; 52(4): 281-286, 2020.
Article in English | MEDLINE | ID: mdl-33165878

ABSTRACT

INTRODUCTION: ANCA-associated vasculitides (AAV) is a group of rare disorders where inflammation and damage of the small blood vessels lead to dysfunction of the supplied organs. In severe flares of the disease patients may require intensive care unit (ICU) admission and treatment. The study aims to characterize Polish patients with AAV who were admitted to the ICU and compare them to the others. MATERIAL AND METHODS: An observational, retrospective study based on the POLVAS - registry of Polish adult patients with AAV was carried out. Patients admitted to the ICU (ICU group) were identified and compared with the patients who did not require ICU admission (non-ICU group). Characteristics and comparison between groups were made using standard statistic descriptive methods. RESULTS: 30 patients admitted to the ICU were identified among 573 cases included in the registry. All patients in the ICU group with available data were ANCA positive. The clinical manifestations related to the ICU admission were respiratory, renal and central nervous system involvement. The treatment regimen for remission induction was similar in both groups. Almost half of the patients in the ICU-group (48.3%) required dialysis, whereas in the non-ICU group it was 21.8% (P = 0.01). Infections were also more frequent in the ICU group (72.4% vs. 36.9% P < 0.001). The mortality rate among patients who needed ICU treatment was significantly higher when compared to the rest of the patients (53.6% vs. 7.8%; P < 0.001). CONCLUSIONS: In the Polish AAV cohort one in twenty patients required ICU admission. This group was characterized by multiple organ involvement and high mortality.


Subject(s)
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/therapy , Adult , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/diagnosis , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/epidemiology , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/mortality , Female , Humans , Intensive Care Units , Male , Registries , Retrospective Studies
7.
Adv Med Sci ; 65(1): 156-162, 2020 Mar.
Article in English | MEDLINE | ID: mdl-31958704

ABSTRACT

PURPOSE: The aim of this study is to present the treatment modalities and associated side effects in a Polish nation-wide ANCA-associated vasculitides (AAV) patients' cohort. MATERIALS AND METHODS: Retrospective analysis of patients diagnosed with AAV between 1990 and 2016, included in the POLVAS registry was performed. Standard descriptive statistic methods were used with an emphasis on the treatment modalities. RESULTS: There were 625 patients diagnosed with AAV included in this study: 417 cases of granulomatosis with polyangiitis (GPA; 66.7%), 106 cases of microscopic polyangiitis (MPA; 17.0%) and 102 cases of eosinophilic granulomatosis with polyangiitis (EGPA; 16.3%). The mean age at the date of diagnosis was 50.4 (±15.7) years and the median observational period amounted to 4.0 (2.0-8.0) years. Glucocorticosteroids (GCs) were the medicaments most frequently used for remission induction (593/622; 95.3%), followed by cyclophosphamide (487/622; 78.3%), rituximab (44/622; 7.1%), and methotrexate (39/622; 6.3%). GCs were also most frequently administered for maintenance therapy (499/592; 84.3%), followed by azathioprine (224/592; 37.8%), methotrexate (136/592; 23.0%) and mycophenolate mofetil (99/592; 16.7%). The median cumulative doses of cyclophosphamide and rituximab equalled 7.99 g (4.18-14.0) and 2000 mg (1500-2800), respectively. The most commonly observed adverse events included: infections - 214/551 cases (38.8%), which were associated with the time of observation (OR = 1.05; 95% CI 1.01-1.10), the use of GCs intravenous pulses (OR = 2.76; 95% CI 1.68-4.54) and need for haemodialysis (OR = 1.73; 95% CI 1.10-2.71). CONCLUSIONS: Polish patients with AAV were predominantly treated according to appropriate guidelines. The most frequent adverse events were typical for usually administered immunosuppressive treatment.


Subject(s)
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/drug therapy , Drug-Related Side Effects and Adverse Reactions/diagnosis , Immunosuppressive Agents/adverse effects , Registries/statistics & numerical data , Adult , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/pathology , Azathioprine/adverse effects , Cyclophosphamide/adverse effects , Drug Therapy, Combination , Drug-Related Side Effects and Adverse Reactions/epidemiology , Drug-Related Side Effects and Adverse Reactions/etiology , Female , Follow-Up Studies , Glucocorticoids/adverse effects , Humans , Male , Methotrexate/adverse effects , Middle Aged , Poland/epidemiology , Prognosis , Retrospective Studies , Rituximab/adverse effects , Survival Rate
8.
Wiad Lek ; 72(9 cz 1): 1691-1696, 2019.
Article in Polish | MEDLINE | ID: mdl-31586985

ABSTRACT

Antineutrophil cytoplasmic antibodies (ANCA) play a key role in the pathogenesis of ANCA-associated vasculitides (AAV). These diseases are rare, occur in every age, but most commonly in adults and elder patients. Among them there are: granulomathosis with poyangiitis (GPA), microscopic poyangiitis (MPA) and eosinophilic granulomathosis with polyangiitis (EGPA). In the article we try to analyse the course of AAV in eldery patients, according to accessible literature. Among AAV patients, those with MPA diagnosis are elder than GPA and EGPA patients. Elder AAV patients present more frequently severe kidney and lung involvement. Elder patients are more at risk to develop complications in the course of disease, but also treatment-related, including severe infections. In elder patients immunosupresive agents dosage, therethore, should be tapered and adjusted to the renal function.


Subject(s)
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/diagnosis , Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis/physiopathology , Aged , Antibodies, Antineutrophil Cytoplasmic/blood , Granulomatosis with Polyangiitis , Humans , Microscopic Polyangiitis
9.
Wiad Lek ; 71(1 pt 1): 17-20, 2018.
Article in Polish | MEDLINE | ID: mdl-29558347

ABSTRACT

OBJECTIVE: Introduction: Musculoskeletal symptoms are not characteristic for granulomatosis with polyangiitis (GPA) patients. However, they could be present in two-thirds of patients at the onset of the disease and may provoke diagnostic difficulties. The aim: To assess retrospectively musculoskeletal manifestations in a group of GPA patients. PATIENTS AND METHODS: Material and methods: The analyzed group consisted of 44 patients with GPA (27M, 17F) aged 46.7 years (16-75) and treated at the Department of Rheumatology and Connective Tissue Diseases in Lublin between 2010-2016. All patients were c-ANCA positive, and p-ANCA were present only in one patient (2.3%). The delay in GPA diagnosis averaged 15.9 months (0-166). RESULTS: Results: Joint manifestations were present in 43.2 % of patients (non-destructive arthritis in 22.7%, arthralgia in 20.5%), in 73.7% of cases anticipating GPA diagnosis by on average of 20.1 months (4-98). In 57.5% of patients joint manifestations were present from the beginning of the disease, most commonly as polyarthritis (68.4%). Myalgia was reported by 11.4% of patients. Among patients with arthritis 5 were RF-positive, none ACPA-positive, and 1 had ANA present. CONCLUSION: Conclusions: Musculoskeletal manifestations appear in a high proportion of GPA patients, in most cases preceding the diagnosis. Such a situation may cause diagnostic difficulties and the diagnosis delay.


Subject(s)
Arthralgia/etiology , Arthritis/etiology , Granulomatosis with Polyangiitis/complications , Myalgia/etiology , Adolescent , Adult , Aged , Antibodies, Antineutrophil Cytoplasmic/blood , Female , Granulomatosis with Polyangiitis/blood , Granulomatosis with Polyangiitis/immunology , Humans , Male , Middle Aged , Retrospective Studies , Young Adult
10.
Case Rep Otolaryngol ; 2016: 5206170, 2016.
Article in English | MEDLINE | ID: mdl-27493820

ABSTRACT

Granulomatosis with polyangiitis is autoimmune and rare disease. It affects many organs, but the most often affected organs are the nose, lungs, and kidneys. It is part of vasculitis and causes an autoimmune attack by an abnormal type of circulating antibody termed ANCAs against small blood vessels. Disease concerns both men and women with a peak age of presentation in the sixth and seven decades. Typically upper and lower respiratory tract and kidneys are involved. Otitis externa, otitis media, or mastoiditis rarely occurs in granulomatosis with polyangiitis. Deafness is the most dangerous aural complication. Histological examination of biopsy is often not specific. A case of GPA with bilateral otitis media, bilateral deafness, and bilateral facial palsy with fatal course is presented.

11.
Wiad Lek ; 69(1): 24-6, 2016.
Article in Polish | MEDLINE | ID: mdl-27162291

ABSTRACT

a-Ku are rare antibodies, which are reported in course of connective tissue diseases. Their prevalence ranges from 0 to 10% , 2%, on average. The main symptoms associated with the presence of a-Ku antibodies include: myositis, arthritis, Raynaud`s phenomenon and skin lesions. The above features are often defined as autoimmune clinical syndrome associated with a-Ku antibodies. In recent years, three cases with the presence of a-Ku antibodies were observed at the Department of Rheumatology and Connective Tissue Diseases. Case 1, a 77-year-old man, with the diagnosis of mixed connective tissue disease according to Raynaud`s phenomenon, myositis, arthritis and presence of a-ribonucleoprotein antibodies. Moreover, secondary Sjögren syndrome (SS) and myasthenia gravis were diagnosed. Case 2, a 56-year-old woman with longstanding history of Raynaud`s phenomenon, sclerodactyly, myositis and arthritis. Based on clinical manifestations and additional tests, systemic sclerosis and myositis were diagnosed. Case 3, a 46-year-old woman with SS diagnosis, long-standing history of Raynaud`s phenomenon, arthralgia and polyneuropathy. Moreover, HCV infection with the presence of cryoglobulin was confirmed. The presence of a-Ku antibodies in high titers was found in all cases. The clinical conditions improved after steroid and immunosuppressive therapy. In conclusion, clinical syndromes with the presence of a-Ku antibodies are associated with a wide range of non-specific symptoms, regarding muscle, joint and skin involvement, in particular. The conditions are more often diagnosed in the elderly; in the majority of cases, they are characterized by mild courses, good response to steroid therapy and good prognosis.


Subject(s)
Antibodies, Antinuclear/blood , Antigens, Nuclear/immunology , Autoimmune Diseases/diagnosis , Autoimmune Diseases/immunology , DNA-Binding Proteins/immunology , Mixed Connective Tissue Disease/diagnosis , Mixed Connective Tissue Disease/immunology , Adult , Aged , Antibodies, Antinuclear/immunology , Arthralgia/etiology , Female , Humans , Ku Autoantigen , Male , Middle Aged , Raynaud Disease/etiology
12.
Pol Arch Med Wewn ; 125(7-8): 538-44, 2015.
Article in English | MEDLINE | ID: mdl-26075796

ABSTRACT

INTRODUCTION: The prevalence of late-onset systemic lupus erythematosus (SLE) diagnosed in patients over the age of 50 years is estimated at 10% to 20%. SLE in elderly patients has specific features with misleading signs and symptoms, but its clinical course seems milder compared with that in younger patients. OBJECTIVES: The aim of the study was to assess clinical manifestations of late-onset SLE in a group of patients treated in Poland. PATIENTS AND METHODS: From a group of 230 consecutive patients with SLE, individuals with late-onset disease were selected. We retrospectively analyzed the incidence of clinical features of SLE, concomitant diseases, and treatment. The incidence of clinical features in late-onset patients was compared with that in a group of 108 patients with early-onset SLE. RESULTS: Late-onset SLE was confirmed in 20 patients (8.7%) including 16 women (80%) and 4 men (20%). A delay in diagnosis was 31.7 months (0-144). The most common SLE features were arthritis (50%), rash (40%), nephropathy (40%), photosensitivity (30%), mouth ulcerations (30%), interstitial lung disease (30%), fever (25%), leukopenia (65%), and thrombocytopenia (35%). Kidney involvement was present in all men and in 25% of women. Thrombotic complications were noted in 38.8% of the patients. Concomitant diseases were common in our study group. CONCLUSIONS: The clinical picture of late-onset SLE differs from that of early-onset SLE. Arthritis, leukopenia, and thrombotic complications are frequent, while skin manifestations, photosensitivity, nephropathy, vasculitis, and central nervous system involvement are less common in late-onset SLE. The diagnosis of late-onset SLE is often delayed, and treatment is determined by the presence of concomitant diseases.


Subject(s)
Lupus Erythematosus, Systemic/pathology , Age of Onset , Aged , Arthritis/complications , Delayed Diagnosis , Exanthema/complications , Female , Humans , Kidney Diseases/complications , Leukopenia/complications , Lung Diseases, Interstitial/complications , Lupus Erythematosus, Systemic/complications , Lupus Erythematosus, Systemic/diagnosis , Male , Middle Aged , Poland , Retrospective Studies , Thrombocytopenia/complications
13.
Wiad Lek ; 68(1): 104-7, 2015.
Article in Polish | MEDLINE | ID: mdl-26094343

ABSTRACT

Still's disease and systemic juvenile idiopathic arthritis (JIA) are multisystem inflammatory diseases of unknown etiology, different disease course and prognosis. Still's disease is characterized by hectic fever, arthritis, skin rash, organomegaly, elevated serum ferritin and inflammatory factors. Early diagnosis and intensive treatment can prevent disease progression and reduce complications such as amyloidosis, physical disability. The first choice of treatment are high doses of corticosteroids and synthetic disease-modifying drugs (DMARDs), including methotrexate (MTX), cyclosporine (CsA). Biologic agents are second line therapy when DMARDs aren't effective, e.g. monoclonal antibodies blocking the action of TNF-alpha (anti-TNF-α), interleukin-1 (ANK--anakinra) and interleukin-6 (TCZ--tocilizumab). We describe in details treatment strategies applied in a young woman with severe Still's disease treated with combination therapy of DMARDs and anti-TNF-α, including etanercept (ETA) or certolizumab (CER). TCZ was applied for the treatment of Still's disease following treatment failure with anti-TNF-α. We've achieved a complete remission of the Still's disease during treatment TCZ.


Subject(s)
Antibodies, Monoclonal, Humanized/administration & dosage , Antirheumatic Agents/therapeutic use , Arthritis, Juvenile/drug therapy , Immunoglobulin Fab Fragments/administration & dosage , Polyethylene Glycols/administration & dosage , Tumor Necrosis Factor-alpha/antagonists & inhibitors , Adult , Antibodies, Monoclonal/therapeutic use , Certolizumab Pegol , Disease Progression , Drug Therapy, Combination , Etanercept , Female , Humans , Immunoglobulin G/administration & dosage , Interleukin-6/antagonists & inhibitors , Methotrexate/administration & dosage , Receptors, Tumor Necrosis Factor/administration & dosage , Remission Induction , Treatment Outcome , Young Adult
14.
Postepy Hig Med Dosw (Online) ; 66: 568-73, 2012 Aug 22.
Article in Polish | MEDLINE | ID: mdl-22922157

ABSTRACT

The prevention of chronic organic damage and complete inhibition of inflammatory activity of the disease are the main goals in the treatment of systemic lupus erythematosus (SLE). Current therapies of SLE are not effective enough and they may cause various serious side effects. Biological therapies, affecting important pathogenetic disturbances in the immunological system of SLE patients, give hope for the development of a new treatment for SLE. Currently the most advanced clinical trials are being conducted with anti-lymphocyte B drugs, such as rituximab, belimumab and epratuzumab. Belimumab as the first biological agent was registered for treatment of the active, seropositive form of SLE. The advances in immunology and rheumatology nowadays raise the hope of finding effective and safe treatment for SLE. In our article we present an overview of data concerning perspectives of biological treatment in SLE.


Subject(s)
B-Cell Activating Factor/antagonists & inhibitors , Biological Therapy , Lupus Erythematosus, Systemic/drug therapy , Antibodies, Monoclonal/therapeutic use , Antibodies, Monoclonal, Humanized/therapeutic use , Antibodies, Monoclonal, Murine-Derived/therapeutic use , Humans , Recombinant Fusion Proteins/therapeutic use , Rituximab
15.
Pol Arch Med Wewn ; 122(10): 480-6, 2012.
Article in English | MEDLINE | ID: mdl-22910193

ABSTRACT

INTRODUCTION: It is still unclear how important the presence of antiphospholipid antibodies (aPL) is in patients with rheumatoid arthritis (RA). OBJECTIVES: The aim of the study was to assess the prevalence of selected aPL in RA patients and their correlation with the presence of markers for RA antibodies and with disease activity. PATIENTS AND METHODS: The study group consisted of 97 patients with RA who had never been treated with biological agents. In all patients, serum anticardiolipin antibodies (aCL), anti­ß2­glycoprotein I antibodies (a­ß2­GPI), lupus anticoagulant (LAC), immunoglobulin M (IgM) rheumatoid factor (RF), and anticyclic citrullinated peptide antibodies (anti­CCP) were measured and disease activity was assessed. RESULTS: The presence of aPL was observed in 27 patients (27.8%): aCL in 20 patients (20.6%), a­ß2­GPI in 12 patients (12.4%), and LAC in 1 patient (1%). Positive aCL of low or medium levels were detected in the IgM class in 11 patients (11.3%) and in the IgG class in 12 patients (12.5%). Positive a­ß2­GPI of low and medium levels were found only in the IgM class. The presence of LAC was associated with aCL­IgM and a­ß2­GPI­IgM. A significant correlation was observed between the presence of anti­CCP and different types of aPL. There was no correlation between aPL and IgM­RF or disease activity markers. CONCLUSIONS: The prevalence of aPL in patients with RA is relatively high. There is a relationship between the prevalence of aPL and anti­CCP-serological marker of RA, but there are no significant correlations between disease activity and the presence of aPL.


Subject(s)
Antibodies, Antiphospholipid/blood , Antibodies, Antiphospholipid/immunology , Arthritis, Rheumatoid/blood , Arthritis, Rheumatoid/immunology , Adult , Aged , Aged, 80 and over , Antibody Formation , Biomarkers/blood , Female , Humans , Male , Middle Aged , Young Adult
16.
Ann Acad Med Stetin ; 57(3): 28-31; discussion 31-2, 2011.
Article in Polish | MEDLINE | ID: mdl-23383545

ABSTRACT

INTRODUCTION: Patients with systemic lupus erythematosus (SLE) demonstrate a significantly greater risk of malignant disease (MD) in comparison to the healthy population. Hematological malignancies, especially lymphomas, predominate among MD patients. It is believed that immune disturbances in SLE, lymphotropic virus superinfections, and long-term immunosuppressive therapy may induce the process of MD. CASE REPORTS: Case 1. A female with a 34-year history of SLE was treated with low doses of glucocorticoids and chloroquine. In July 2011, severe pancytopenia complicated by septic shock was observed and the patient died. Histopathologic examination of bone marrow revealed the presence of a B-cell lymphoma. Case 2. A female with a 26-year history of SLE presented with anti-dsDNA and anti-SSA antibodies. Exacerbations of SLE were treated with high doses of glucocorticoids, cyclophosphamide, and azathioprine. In December 2011, pancytopenia was observed and bone marrow histology was in favor of acute monoblastic leukemia. CONCLUSIONS: Data from the literature and our own observations confirm the necessity of oncologic follow-up in SLE. The risk of MD in SLE increases with duration of the disease and depends on the serologic profile of SLE and medication used. Hematological exacerbations in SLE, particularly protracted and therapy-resistant anemia and leukopenia, should be differentiated from a hematological malignancy.


Subject(s)
Hematologic Neoplasms/etiology , Leukemia, Monocytic, Acute/etiology , Leukemia, Monocytic, Acute/pathology , Lupus Erythematosus, Systemic/complications , Lymphoma, B-Cell/etiology , Lymphoma, B-Cell/pathology , Aged , Fatal Outcome , Female , Humans , Lupus Erythematosus, Systemic/drug therapy , Pancytopenia/etiology , Shock, Septic/etiology
17.
Rheumatol Int ; 28(9): 919-23, 2008 Jul.
Article in English | MEDLINE | ID: mdl-18320193

ABSTRACT

We report the case of coexistence of five autoimmune diseases in a 36-year-old woman, who initially developed psoriasis. Several years later, the patient was diagnosed with a mixed connective tissue disease and primary biliary cirrhosis (PBC). On admission to the Department of Rheumatology and Connective Tissue Diseases, the patient fulfilled classification criteria of an overlap syndrome systemic lupus erythematosus (SLE) with secondary antiphospholipid syndrome/systemic sclerosis (SSc)/Sjogren's syndrome (SS) with coexisting PBC and psoriasis. The SLE symptoms included discoid lupus erythematosus, arthritis, pancytopenia, antinuclear antibodies and anticardiolipin antibodies. Moreover, the patient met the criteria of antiphospholipid syndrome diagnosed based on preterm delivery before week 34, and high values of anticardiolipin antibodies were found at repeated determinations. The SSc symptoms included sclerodactyly, pulmonary fibrosis with pulmonary hypertension and esophageal dysfunction. The SS syndrome involved xerostomia, xerophthalmia, the positive Schirmer's test and presence of anti-SS antibodies. The literature reports overlap syndromes in various combinations; however, the coexistence of five autoimmune diseases is extremely rare.


Subject(s)
Connective Tissue Diseases/complications , Connective Tissue Diseases/diagnosis , Liver Cirrhosis, Biliary/complications , Adult , Connective Tissue Diseases/drug therapy , Cyclophosphamide/administration & dosage , Drug Administration Schedule , Female , Humans , Immunosuppressive Agents/administration & dosage , Syndrome
18.
Pol Arch Med Wewn ; 112(1): 805-9, 2004 Jul.
Article in Polish | MEDLINE | ID: mdl-15526840

ABSTRACT

Surface phenomena resulting from interactions between molecules occur commonly in nature. Peritoneal effluent is a mixture of organic and inorganic substances both macro- and micromolecular. Surfactants present in dialysate affect its surface properties. Among them are: proteins, phospholipids, fatty acids. Our aim in this study was to investigate relationships between peritoneal membrane solute transport characteristics and surface tension of peritoneal effluent. The study was conducted in 40 CAPD patients who were stable, without peritonitis (24 M, 16F), age 51.5 +/- 15.8 (range 30-79) mean CAPD duration 26.4 +/- 20.6 months (range 4-72). Standard peritoneal equilibration test (sPET) was done in all patients. Dialysate surface tension (ST) values after 4 hours dewell were determined using Wilhelmy Plate method. Mean ST values of individual dialysate sample based on 10 measurements were calculated. According to the PET values patients were divided into two groups: group 1 (high/high average transporters, n = 26) and group 2 (low average/low transporters, n = 14). Patients in group 1 had significantly lower ST of dialysate than patients in group 2 (51.2 +/- 4.8 vs 57.9 +/- 1.4 mN/m), p<0.01. The lowest values of ST (48.5 +/- 5 mN/m) were found in patients classified as high transporters (n = 8). Correlation's: significant negative correlation was found between ST and D4/P4 for creatinine (r = -0.45, p<0.005) and significant positive correlation between ST and D4/DO for glucose (r = 0.48, p = 0.003). We conclude that there are significant relationships between peritoneal transport status and surface tension of peritoneal effluent. High transporters have significantly higher concentrations of surfactants in dialysis effluent.


Subject(s)
Dialysis Solutions/metabolism , Peritoneal Dialysis, Continuous Ambulatory , Surface-Active Agents/metabolism , Adult , Aged , Biological Transport , Fatty Acids/metabolism , Female , Humans , Male , Middle Aged , Phospholipids/metabolism , Proteins/metabolism , Surface Tension , Time Factors
19.
Article in English | MEDLINE | ID: mdl-12898852

ABSTRACT

Surface phenomena resulting from interactions among molecules occur commonly in nature. Surfactants, substances with surface activity, have a big influence on surface tension. They reduce surface tension when added to the solution, even in minimal concentration. The behaviour of surface tension of the dialysate is difficult to properly assess, because of its complex composition. Dialysate is a mixture of organic and inorganic substances, both macro- and micromolecular. Among them are proteins, phospholipids, fatty acids. In the literature there are no publications showing the behaviour of surface tension of the dialysate. The aim of the study was the assessment of changes of dialysate surface tension in non-complicated continuous peritoneal dialysis (CAPD), depending on time of the presence of dialysis fluid in the peritoneal cavity and on the concentration of different substances in it. The study was performed on 39 dialysate samples obtained from 6 patients chronically treated with continuous ambulatory peritoneal dialysis (CAPD). During the study patients had no symptoms of peritonitis. Surface tension of the dialysate was determined, using the Wilhelmy's method. Ten measurements were performed in every sample of the dialysate and the mean values and standard deviation were calculated. Simultaneously the concentrations of sodium, glucose, urea, creatinine, total protein were measured in the dialysate. As a result of the study a significant negative correlation between dialysate protein concentration and surface tension was found. The significant negative correlation between surface tension and the dwell time was also found. There was no significant correlation between the value of surface tension of the dialysate and concentrations of glucose, urea, creatinine and sodium.


Subject(s)
Dialysis Solutions/analysis , Dialysis Solutions/chemistry , Peritoneal Dialysis, Continuous Ambulatory , Creatine/analysis , Glucose/analysis , Humans , Proteins/analysis , Sodium/analysis , Surface Tension , Urea/analysis
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