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Abstract

Goodpasture syndrome is a rare autoimmune disease, with significant morbidity and mortality in young people and otherwise healthy population. Complete disease remission is possible with prompt diagnosis and treatment. We report 3 cases with Goodpasture syndrome treated at the Department of Nephrology, University Clinic of Nephrology, with different outcome. All of the patients were with similar clinical feature, with renal failure that needed treatment with hemodialysis. But results of the treatment with plasmapheresis indicate that this procedure reduces morbidity in patients with Goodpasture syndrome. The clinical course and the outcome of the disease were different. The disease is unpredictable, and the early diagnosis and start with the treatment is important for the remission.

of plasma exchange and immunoadsorption in systemic lupus erythematosus and antiphospholipid syndrome: A systematic review. Autoimmunity Reviews 15 (2016) 38–49 15. Zhang YY, Tang Z, Chen DM, Gong DH, Ji DX, Liu ZH. Comparison of double filtration plasma-pheresis with immunoadsorption therapy in patients with anti-glomerular basement membrane nephritis. BMC Nephrol 2014;15:128. 16. Derksen RH, Hene RJ, Kallenberg CG, Valentijn RM, Kater L. Prospective multicentre trial on the short-term effects of plasma exchange versus cytotoxic drugs in steroidresistant lupus

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a clinical syndrome that manifests with thrombocytopenia, microangiopathic haemolytic anaemia and symptoms and signs of kidney and brain damage, but it rarely involves other organs. The main pathophysiological cause of TTP is diminished metalloproteinase ADAMTS13 activity; the main function of ADAMTS13 is to degrade large multimers of the von Willebrand factor. Diminished activity of ADAMTS13 is caused either by a genetic mutation in the gene that codes ADAMTS13 (congenital TTP) or by antibodies that block ADAMTS13 enzyme activity or accelerate the degradation of ADAMTS13 (acquired TTP). Clinically, TTP presents most frequently with signs and symptoms of brain and kidney damage with concomitant haemorrhagic syndrome. TTP is suspected when a patient presents with a low platelet count, microangiopathic haemolytic anaemia (negative Coombs tests, low haptoglobine concentration, increased serum concentration of indirect bilirubin and lactate dehydrogenase, increased number of schysocytes in peripheral blood) and the typical clinical presentation. A definitive diagnose can be made only by measuring the ADAMTS13 activity. The differential diagnosis in such cases includes both typical and atypical haemolytic uremic syndrome, disseminated intravascular coagulation, HELLP syndrome in pregnant women and other thrombotic microangiopathies. The first line therapy for TTP is plasma exchange. In patients with acquired TTP, in addition to plasma exchange, immunosuppressive medications are used (corticosteroids and rituximab). In patients with hereditary TTP, the administration of fresh frozen plasma is sometimes required.

Abstract

Hemolytic-Uremic Syndrome (HUS) is a clinical syndrome with a triad of non-immune Microangiopathic Hemolytic Anemia (MAHA), thrombocytopenia and renal failure. Together with the Thrombotic Thrombocytopenic Purpura (TTP), it belongs to a group of diseases characterized as the Thrombotic Microangiopathy (TMA), which represents a microvascular occlusive disorder with the formation of a predominantly thrombocytic thrombus in the renal and/or systemic circulation. In the period starting from 2001 to 2017, 14 patients with a HUS were diagnosed at the Clinic for Nephrology (unfortunately ADAMTS 13 could not have been done due to technical reasons). In a retrospective clinical laboratory analysis and monitoring, we obtained the following results. Out of 14 patients, 10 were female (or 71.43%) and 4 were male (28.57%), the youngest patient was aged 17 and the oldest one 78, the average age of our patients was 55.33 years, the annual number of patients with the diagnosis of HUS was 0.93 patients per year, or 0.00116 in relation to the total number of patients treated. After monitoring the patients individually for the period ranging from 1 to 14 years, a stable remission was achieved in 5 patients, while a chronic renal insufficiency occurred in 3 patients. In two of our patients, a percutaneous kidney biopsy was performed with pathohistological findings described in references. Having done this retrospective analysis, we can conclude that the survival and complications of this rare, but serious disease correspond to the available world data.

. Plasmapheresis for the treatment of kidney diseases. Kidney Int 2016; 90(5): 974-84. 22. Andries G, Karass M, Yandrapilli S, Linder K, Liu D, Nelson J, et al. Atypical hemolytic uremic syndrome in first trimester pregnancy successfully treated with eculizumab. ExpHematolOncol 2017; 6: 4. Doi: 10.1186/s40164-017-0064-7. 23. De Sousa-Amorim E, Pelicano MB, Quintana LF, Campistol JM. Eculizumab in pregnancy-associated atypical hemolytic uremic syndrome: insights for optimizing management. J Nephrol 2015; 28(5): 641-5. 24. Zuber J, Fakhouri F, Roumenina LT, Loirat C, Fremeaux

, Harrison RA et al. Risks and benefits of omega 3 fats for mortality, cardiovascular disease and cancer: Systematic review. BMJ 332: 752-760, 2006. 21. Shearer GC, Savinova OV, Harris WS. Fish oil-how does it reduce plasma triglycerides? Biochim Biophys Acta 1821(5): 843-851, 2012. 22. Nasa P, Alexander G, Kulkarni A et al. Early plasmapheresis in patients with severe hypertriglyceridemia induced acute pancreatitis. Indian J Crit Care Med 19(8): 487-489, 2015. 23. Stefanutti C, Di Giacomo S, Vivenzio A et al. Therapeutic plasma exchange in patients with severe

immunoglobulins and topical conservative approach: a retrospective analysis. Burns. 2007;33:452-9. 35. Brown KM, Silver GM, Halerz M, Walaszek P, Sandroni A, Gamelli RL. Toxic epidermal necrolysis: does immunoglobulin make a difference? J Burn Care Rehabil. 2004;25:81-8. 36. Chaidemenos GC, Chrysomallis F, Sombolos K, Mourellou O, Ioannides D, Papakonstantinou M. Plasmapheresis in toxic epidermal necrolysis. Int J Dermatol. 1997;36:218-21. 37. Lissia M, Figus A, Rubino C. Intravenous immunoglobulins and plasmapheresis combined treatment in patients with severe toxic epidermal

Abstract

Rapidly progressive glomerulonephritides are relatively rare but serious disorders of diverse etiology, which share some clinical features: rapid evolution, progressive to renal failure, often accompanied by oliguria or anuria. They are characterized histopathologically by an intense extracapillary proliferation, with the development of crescents (semilunar lesions) in over 50% of examined glomeruli. The following pathological entities are referred to as rapidly progressive glomerulonephritides: ANCA-positive pauci-immune vasculitides (microscopic polyangiitis, granulomatosis associated with microscopic polyangiitis, allergic granulomatosis associated with microscopic polyangiitis), extracapillary proliferative glomerulonephritides by immune complexes and glomerulonephritides by anti-glomerular basement membrane antibodies. Due to major histopathological and functional complications, their evolution to death or renal replacement therapy occurs within 6-2 months after the diagnosis, if they are not treated, but the evolution is favorably influenced by aggressive immunosuppression, whether or not associated with plasmapheresis.

Abstract

Background. Sjogren’s syndrome (SS) is among the most frequent autoimmune diseases and one of its most severe extraglandular manifestations is peripheral neuropathy. There is no consensus about peripheral neuropathy treatment in SS. Our aim is to identify studies proving the efficiency of immunosuppressive treatment on peripheral neuropathies in SS.

Methods. The search was conducted on the PubMed (MEDLINE) database. Studies with patients diagnosed with SS and peripheral neuropathy were included. Treatment with one of the following was among inclusion criteria: glucocorticoids (GC), rituximab (RTX), azathioprine (AZA), mycophenolic acid (MMF), cyclophosphamide (CP), methotrexate (MTX), plasmapheresis or iv immunoglobulins (IV IG).

Results. A total of 116 results were found and abstracts were examined. 103 papers were excluded, and the remaining 13 papers were analyzed. They were 3 case series and 10 case reports, retrospective, totalizing 62 patients of which 22 (35.5%) received IV IG, 8 (13%) received RTX, 7 (11%) CP, and 5 (8%) received only GC. Drug associations containing corticosteroids were frequent. Of those 22 treated with IV IG, 18 patients improved (82%), and 4 stabilized (18%).

IV IG was useful in sensory, motor and sensorimotor neuropathies. CP had good results in mononeuritis multiplex, while autonomic neuropathies responded well to GC or RTX. AZA, RTX, MTX, MMF or plasmapheresis were not used alone. Follow-up periods were heterogenous and the evaluation of the neuropathy was not systematic.

Conclusion. There is only low level evidence (retrospective case reports and case series). In most cases, IV IG treatment in patients with peripheral neuropathies and SS resulted in clinical improvement, while other therapies, such as RTX, corticosteroids and CP proved to be useful in a handful of cases.

Abstract

Granulomatosis polyangiitis (Wegener's granulomatosis) is an ANCA-associated necrotising vasculitis. The disease involves upper respiratory tract, the lungs and kidneys but central nervous system (CNS) involvement is 1-5%.

A 40-year-old male patient was admitted to the hospital with joint pain, rash, aphthous lesions. The skin biopsy from the lesion showed leukocytoclastic vasculitis. The patient had c-ANCA positive and was diagnosed granulomatosis polyangiitis. He was treated with a pulse steroid and cyclophosphamide. Before the 5th session of therapy, the patient developed hemoptysis and hematuria. Thorax CT (computarized tomography) showed a diffuse alveolar hemorrhage and hence plasmapheresis and IVIG (intravenous immunoglobulin) were added to the treatment. Two days after IVIG, the patient developed globe vesical, headache and respiratory arrest. MR (magnetic resonance) showed CNS involvement. The patient was treated with a pulse steroid, but did not respond to therapy and died after 5 months since establishing the diagnosis.

More studies are needed to identify effective treatment and course of disease for patients with central nervous system involvement.