Myositis-specific autoantibodies in a non-traveler, patient from a non-endemic country, with Plasmodium vivax malaria.

INTRODUCTION: Autoantibodies (AAb) are a hallmark of immune-mediated inflammatory diseases. Malaria is a parasitic disease caused by Plasmodium protozoa. Individuals with malaria may present with a wide range of symptoms. It is frequently linked to the development of different AAb. CASE DESCRIPTION: A 35-year-old male presented with repeated episodes of fever, malaise, myalgia, dark urine, and yellowish sclera. Initial diagnostic workup revealed severe Coombs-positive anemia, increased C-reactive protein, and procalcitonin, pathological liver tests, high concentration of serum IgE, IgG, IgM, IgA, positive antinuclear antibodies (ANA), and positive antineutrophil cytoplasmatic antibodies (ANCA). In addition, myositis-specific antibodies directed to polymiositis-scleroderma 75 protein (PmScl75), threonyl-tRNA synthetase (PL-7), alanyl-tRNA synthetase (PL-12), Mi-2 antigen (Mi-2), Ku DNA helicase complex (Ku), signal recognition particle (SRP), and antiaminoacyl tRNA synthetase (EJ) were detected. The patient was suspected of having systemic lupus erythematosus and sent to the Clinic of Allergy and Immunology for further evaluation and treatment. A peripheral blood film examined by the hematologist during an episode of fever revealed intra-erythrocytic parasitic forms of Plasmodium vivax (P. vivax). After being diagnosed with P. vivax malaria, he was transferred to the Clinic for Infective and Tropical Diseases. The therapy consisted of artesunate/mefloquine and prednisone led to a complete clinical recovery and autoantibodies gradually disappeared. CONCLUSIONS: Malaria would not normally be considered during the initial diagnostic workup in a non-traveler and a patient from a non-endemic country. However, a thorough parasitic evaluation in patients presenting with a broad range of autoantibodies might be of particular importance.

Comprehensive Analysis of the HLA Class I and the HLA Class II Alleles in Patients with Takayasu Arteritis: Relationship with Clinical Patterns and Prognosis

BACKGROUND: Takayasu arteritis (TA) is a systemic vasculitis, affecting mainly the aorta and its branches. OBJECTIVE: To analyze the HLA class I and class II alleles in patients with TA and explore their relationship with clinical and demographic characteristics, and potential significance in prognosis. METHODS: Twenty-five, unrelated TA patients were genotyped for HLA-A, HLA-B, HLA-C, HLA-DRB1, and the HLA-DQB1 loci. The frequencies of the HLA-A, HLA-B, and the HLA-DRB1 were compared with a control group of 1992, while the HLA-C and the HLA-DQB1 were compared with a group of 159 healthy, unrelated individuals. RESULTS: Among TA patients, 5/25 (20%) were identified as the HLA-B*52 carriers. There was a significant difference in the HLA-B*52 allele frequency in the TA patients (10%) compared with the healthy controls (1.2%). Moreover, presence of the HLA-B*52 was associated with significantly earlier disease onset, more severe clinical presentations, and a poorer response to treatment. The HLA-C*03 was detected in 32% of patients and was present exclusively in those with a clinically mild form of the TA, indicating a putative protective effect. CONCLUSION: These findings indicate that the HLA-B*52 allele contributes to a higher susceptibility to the TA whereas the HLA-C*03, can be a protective factor in the TA.

Severe strongyloidiasis and systemic vasculitis: comorbidity, association or both? Case-based review.

A possible association between strongyloidiasis and systemic vasculitis is rarely reported in the literature. We report the case of a patient with severe strongyloidiasis and an angiographic finding consistent with polyarteritis nodosa. Diagnosis of strongyloidiasis was made by finding of larvae and adult parasites in samples of the upper gastrointestinal tract mucosa and stool. The patient was treated with albendazole, ivermectin and corticosteroid withdrawal. This therapy led to the resolution of symptoms, with repeated stool samples negative for S. stercoralis. However, the clinical course was complicated with pulmonary tuberculosis. Despite tuberculostatic therapy and supportive measures, a lethal outcome occurred. The report is followed by a focused review of the available literature on the association of strongyloidiasis and systemic vasculitis.

HCV related severe cryoglobulinemic vasculitis treated with plasma exchange and rituximab: case report and literature review.

Mixed cryoglobulinemia is the most prevalent extrahepatic manifestation of chronic HCV infection. It is usually a benign lymphoproliferative disorder which presents as vasculitis affecting different organs. Although life-threatening cryoglobulinemic vasculitis (CryoVas) is rare, it is sometimes the first and possibly lethal complication. Its treatment depends on the severity of vasculitis and can be challenging. High dose of corticosteroids, immunosuppressive agents and plasma exchange represent the first-line treatment, which should be followed by antiviral therapy. Rituximab is an effective and safe treatment option. However, the data about its use in life-threatening conditions are scarce. We report the case of a patient with severe, relapsing and life-threatening HCV-related CryoVas  resistant to standard therapy who had had an initial beneficial response to rituximab added to plasma exchange that was later compromised by the development of sepsis. We also review the literature and discuss manifestations and therapy of life-threatening Cryovas with focus on rituximab use.

Vitamin D Status in Patients with Systemic Lupus Erythematosus in Serbia: Correlation with Disease Activity and Clinical Manifestations.

BACKGROUND: Numerous studies indicate potential role of vitamin D as an important factor in the development of many autoimmune diseases including systemic lupus erythematosus (SLE). Patients with SLE are especially prone to the development of vitamin D deficiency due to the nature of their illness. AIM: The aims of our study were to determine the prevalence of vitamin D insufficiency and deficiency in patients with SLE in Serbia, to identify clinical variables associated with vitamin D status and to examine the impact of vitamin D status on disease activity and presence of specific lupus autoantibodies. MATERIAL AND METHODS: The study included 46 patients with SLE. Serum 25(OH)D concentration was measured by electrohemiluminiscent immunoassay. RESULTS: The mean serum concentration of 25(OH)D was 11.9 ± 7.3 ng/ml. The prevalence of insufficiency was 32.6%, while the prevalence of deficiency was 67.4%. There was no association between vitamin D status and photosensitivity, skin lesions, arthritis and lupus nephritis. Vitamin D status was not associated with the presence of specific autoantibodies. There was no correlation between disease activity assessed by SLEDAI scale with the concentration of 25(OH)D. Patients who used vitamin D supplements and calcium did not have a significantly higher concentration of 25(OH)D. CONCLUSION: In conclusion, vitamin D deficiency is common in patients with SLE.

Sjögren's Syndrome and Silicosis - a Case Report.

Sjögren's syndrome is an autoimmune disease of unknown etiology where immune response to self-antigens is believed to result from interactions between genetic and environmental factors. We describe the case of a patient who has been diagnosed with Sjögren's syndrome based on typical clinical and immunological parameters. The clinical picture was dominated by the respiratory symptoms, and radiographic and multislice computed tomography examination of the chest showed certain changes characteristic of pneumoconiosis. Given that the patient has worked in a foundry where he has been exposed to the silica dust, he was subject to examination by occupational health specialists under the suspicion of lung silicosis, who confirmed the silicosis. This case report points to the possible connection between a professional exposure to silica and Sjögren's syndrome. Occupational exposure to silica is a possible risk factor for the development of autoimmune diseases, and in the evaluation of patients with connective tissue diseases it is important to consider work-related history.

CLINICAL CHARACTERISTICS AND TREATMENT OF MELKERSSON-ROSENTHAL SYNDROME--OVERVIEW OF SIX PATIENTS.

INTRODUCTION: Melkersson-Rosenthal syndrome is a rare disease of unknown etiology. Histopathologically, it presents as granulomatous cheilitis. From laboratory aspect, it is a nonspecific, differential diagnostically and therapeutically complex condition. CASE REPORT: This is a report of six cases treated at the Department of Allergology and Immunology of the Clinical Center of Serbia, who had presented with the referral diagnosis of recurring or persistent lip edema, and who were diagnosed with Melkersson-Rosenthal syndrome upon detailed evaluation. Three patients had complete triad of symptoms, two had the oligosymptomatic form and one manifested the monosymptomatic form of the disease. Histopathological findings of the oral mucosa specimens verified the presence of non-necrotic epithelioid granulomas in all patients. The patients were treated with the H1 and H2 antihistamines, corticosteroids, followed by anabolic drugs and antibiotics, resulting in transient and unfavorable effects. CONCLUSION: In differential diagnosis, Melkersson-Rosenthal syndrome diagnosis primarily refers to conditions of angioneurotic edema and hereditary angioedema, as well as granulomatous diseases such as sarcoidosis, tuberculosis and Chron's disease. It is necessary to follow-up these patients in view of monitoring the effects of the therapy and possible development of systemic granulomatous diseases.

Acute meningoencephalitis in a patient with systemic lupus erythematosus.

INTRODUCTION: Infections in patients with systemic lupus erythematosus (SLE) are a significant factor of morbidity and mortality. Although central nervous system infections, including septic meningitis, are rare in patients with SLE, they can be significant causes of mortality inspite of the prompt and accurate diagnosis and proper management. CASE REPORT: We presented a woman with the diagnosis of SLE and diffuse proliferative lupus nephritis. Because of disease activity we introduced cytostatic immunosuppressive therapy, cyclophosphamide and then azathioprine. Meningoencephalitis, staphylococcal sepsis and abscess of the brain, with resulting seizures developed. CONCLUSION: This case alerts to the need of careful examination of patients with SLE, collection of adequate cultures and evaluation of predisposition towards infections, before the introduction of immunosuppressants due to potentially fatal infection.

[Importance of dysphagia examination in patient with dermatomyositis--case report].

INTRODUCTION: Polymiositis belongs to the group of inflammatory myopathies which are manifested by muscle weakness of the shoulder blade and pelvic region. The presence of typical skin manifestations is suggestive of dermatomyositis. These patients may also develop dysphagia (10-54%) as a result of involvement of the oropharyngeal and upper oesophageal striated muscles. Dermatomyositis may also be associated with another systemic disease or malignancy. CASE REPORT: Hereby is presented the case of a 42-year-old female patient hospitalized at the Department of Allergy and Immunology, Clinical Center of Serbia for the shoulder blade and pelvic muscle weakness and pains in the small and large joints, eyelid edema, facial and neckline redness, difficult swallowing and loss of body mass. Based on the presence of proximal muscle weakness, increased enzyme serum levels (lactic acid dehydrogenase, glutamic-oxalacetic transaminase), positive electroneuromyography findings, typical skin changes and positive muscle biopsy, the patient was diagnosed to have dermatomyositis. Both radioscopy and esophagography revealed some disturbances in all phases of swallowing, absence of all primary and secondary peristaltic waves accompanied by contrast medium aspiration. Additionally, esophageal manometry proved the absence of esophageal peristalsis. Additional examinations ruled out the presence of any malignancies. The patient underwent glycocorticoid and azatioprim treatment along with specific dietary regimen, symptomatic and physical therapy, which led to favorable clinical outcome. CONCLUSION: Dermatomyositis-associated dysphagia may lead to severe complications such as cachexia and aspiration pneumonia. In addition to the management of underlying disease, the treatment includes special dietary regimen, rehabilitation and even interventional surgical procedures, if necessary.

[Laboratory and morphologic parameters in patients with lupus nephritis]. / Laboratorijski i morfoloski parametri kod bolesnika s lupusnim nefritisom.

INTRODUCTION: Lupus nephritis is an example of glomerulonephritis mediated by immune complexes. The information obtained by kidney biopsy corroborates diagnosis and evaluation of disease activity, specify of prognosis and mode of treatment. The object of our study was to determine the prevalence of particular pathohistological types of lupus nephritis in our group of patients, to establish if there was a correlation of laboratory and morphological parameters, and to present the use of specific therapeutical protocols. METHOD: The study included 58 patients with diagnosed systemic lupus erythematosus (SLE) and lupus nephritis, who had biopsy of kidneys. The indications for biopsy were the following: proteinuria level over 0.5 g/24 hrs, erythrocyturia and cylindruria. The patients were examined and treated at the Institute of Allergology and Immunology, Clinical Centre in Belgrade, over the period 1994-2001. Within the testing, besides standard laboratory tests, the immunological evaluation was also performed as follows: the level of standard serum immunoglobulins, C3 and C4 components of complement, antinuclear antibodies (ANA) and antibodies to double-stranded DNA (dsDNA), were determined. RESULTS: There was 84.48% of female patients in the studied group. The mean-age was 36.5 years, while the average duration of disease (SLE) to kidney biopsy was 28.3 months. Considering cytopenia, leukopenia was found in 26.79% of patients, lymphopenia was recorded in 62.26% of cases while anaemia was noted in 52.63% of patients. The values of serum creatinine were elevated in 25.86% of patients, while creatinine clearance rate was lower (below 80 ml/min) in 75% of cases. The values of proteinuria are illustrated in Graph I. Cylindruria was found in 20.69% of subjects, massive erythrocyturia in 44.83%, and 46.55% of patients had more than 5 red blood cells in urinary sediment. Regarding the pathohistological findings, according to WHO classification, the biopsy of kidneys revealed the following distribution: class I--3.45% of patients, class IIA--24.14%, class IIB--31.03%, class III--12.07%, class IV--24.14%, class V--3.35%, and class VI 1.72% of cases. DISCUSSION: Within the immunological evaluation, the increased serum immunoglobulin G (IgG) level was found in 26.79% of subjects, suggesting that the consumption of complements in formation of immune complexes was the basic pathogenetic mechanism of lupus nephritis. Positive finding of ANA was recorded in about 95% of subjects, what was typical for SLE, while antibodies to dsDNA were positive in no less than 72.72% of cases, arguing for the fact that they were one of major nephritogenic antibodies. Considering the correlation analysis, no correlation between pathohistological findings and serum creatinine level was found, but there was the correlation between pathohistological findings and decreased creatinine clearance rate. The correlation between pathohistological findings and proteinuria up to 0.5 g/24 hrs was verified. There was no correlation between the increased IgG level and kidney biopsy findings, but it was found that decreased level of C4 complement component correlated with the degree of kidney lesion. The value of diastolic pressure also correlated with pathohistological findings. Concerning the applied mode of treatment, 46.55% of patients were administered pulse doses of cyclophosphamide combined with pulse doses of methylprednisolone, 5.17% had pulse doses of cyclophosphamide and 32.76% pulse doses of methylprednisolone, while 12.07% received glucocorticoid drugs combined with azathioprine, and glucocorticoids only were given to 3.45% of them. The conclusion will be that the biopsy of kidneys is the imperative in the evaluation of lupus nephritis, because the complete insight into the degree and type of kidney lesion as well as search for an optimal mode of treatment may be achieved only by assessment of combined clinical, laboratory and morphological parameters.