IgA nephropathy and atypical anti GBM disease: A rare dual pathology in a pediatric RPGN

Introduction: Anti-GBM nephritis in the pediatric age group is exceedingly rare with concurrent additional pathologies being even rarer. Tissue diagnosis requires a combination of crescentic histomorphology, immunofluorescence showing “Paint brush stroke” pattern of linear IgG or rarely IgA and serum anti-GBM antibodies subject to the disease course and treatment. The authors describe one such case with a dual pathology involving IgA nephropathy and atypical anti-GBM disease. Case presentation: A thirteen-year-old girl presenting with features of rapidly progressive glomerulonephritis underwent a renal biopsy showing a mesangioproliferative histology with crescents and an immunofluorescence pattern indicating a dual pathology of IgA Nephropathy and Anti GBM Nephritis. Additional ancillary testing including staining for IgG subclasses and galactose deficient IgA (KM55) helped to confirm the diagnosis. She responded to steroid pulses and plasma exchange therapy, was off dialysis after 8 weeks with serum creatinine of 1.5 mg/dl however remains proteinuric at last follow up. Conclusion: Concurrent Anti-GBM nephritis and IgA nephropathy is a rare occurrence and possibly arises from a complex interaction between the anti-GBM antibodies and the basement membrane unmasking the antigens for IgA antibodies. Additional newer techniques like immunofluorescence for KM 55 are helpful in establishing the dual pathology.

A case of Hemophagocytic lymphohistiocytosis induced by COVID-19, and review of all cases reported in the literature

Novel coronavirus infections 2019 (COVID-19) associated hyperinflammatory syndromes are well-defined clinical conditions and have a potential risk for severe infection. Hemophagocytic lymphohistiocytosis (HLH), a rare type of acute progressive hyperinflammatory syndrome, has been reported in a limited number of COVID-19 cases. In this article, we aimed to present a patient with HLH secondary to COVID-19 diagnosed by bone marrow biopsy, and to summarize and review HLH cases associated with COVID-19 in the literature. A 47-year-old male patient presented with complaints of fever, cough, abdominal discomfort, and nausea-vomiting. He had recovered from COVID-19 a month ago and was readmitted to the hospital due to the re-appearance of clinical symptoms after a two-week interval. The patient was diagnosed with HLH secondary to COVID-19 on sixth day of admission and fully recovered with systemic pulse steroid, intravenous immunoglobulin, and plasma exchange therapy. Analysis of literature searches revealed that 22 cases were definitely diagnosed with COVID-19-associated HLH, 16 of them were male. They had been treated with different anti-cytokine drugs, of which nine had died. The increasing number of HLH cases, which have high mortality rates, shows the importance of hyperinflammatory syndromes in COVID-19 patients. Some patients may experience hemophagocytosis in the late period of COVID-19, even while in recovery. Increased awareness and early treatment for HLH triggered by COVID-19 can be a life-saving effort for reducing mortality in severe COVID-19 cases.

Does withdrawal of immunosuppression in rheumatoid arthritis after SARS-CoV-2 infection increase the risk of vasculitis?

We describe a case of a 48-year-old woman who presented with acute respiratory failure due to diffuse alveolar haemorrhage and acute renal failure due to pauci-immune glomerulonephritis consistent with a new diagnosis of microscopic polyangiitis (MPA). The patient had a recent SARS-CoV-2 infection 6 weeks before MPA diagnosis and had stopped immunosuppression for her rheumatoid arthritis (RA) at that time. The patient was treated with pulse intravenous steroids, plasma exchange therapy and rituximab, which induced remission of her illness. This case highlights a timely dilemma of holding immunosuppression in a RA patient with low disease activity on combination therapy with SARS-CoV-2 infection, and the potential risk of developing an additional autoimmune disease, such as vasculitis, given their existing autoimmunity due to RA.

A combined approach to treat heparin-induced thrombocytopaenia before heart transplant

Heparin-induced thrombocytopaenia (HIT) complicates the management of patients in need for mechanical circulatory support awaiting heart transplantation. The limited available treatment options are fraught with complications and limitations in their applicability. We report on the combined use of therapeutic plasma exchange therapy and intravenous immunoglobulin, used in 3 consecutive heparin-induced thrombocytopaenia-positive patients on temporary mechanical circulatory support awaiting urgent heart transplant. This combined approach allowed us to use heparin safely.

AB0444 THROMBOTIC MICROANGIOPATHY AND SJÖGREN SYNDROME, AN UNUSUAL ASSOCIATION. PRESENTATION OF CLINICAL CASE AND SYSTEMATIC REVIEW OF THE LITERATURE

Background:Thrombotic microangiopathy (TMA) is a clinicopathologic diagnosis defined as microangiopathic hemolytic anemia (MAHA) with associated features of thrombocytopenia and end-organ ischemia. Systemic lupus erythematosus, antiphospholipid antibody syndrome, and scleroderma, are within the autoimmune diseases, the more commonly associated with TMA. It has been considered that the association with Sjögren Syndrome (SS) is rare.Objectives:To describe one patient with TMA and SS, and to review all cases reported in the literature.Methods:We notified a clinical case of a patient with Sjögren’s syndrome and TMA. Then, we searched the medical literature finding a total of 17 cases reported with this association until 2019. Before the data obtained were tabulated by trained staff and descriptive, comparing groups and bivariate analysis was performed. The outcome of interest was the death of the patient. Stata 12.0 software was used.Results:A 26-years-old Colombian female presented with a 6-week history of petechiae in lower limbs, gingivorrhagia, menorrhagia and jaundice; and previous history of arthritis and xerostomia. On admission, in the context of severe thrombocytopenia and MAHA, MAT diagnosis was made. During hospitalization we confirm the diagnosis of SS with ANA, antiRo and salivary gland biopsy. The patient was treated with steroids (methylprednisolone 500 mg/day, 3 days), plasma exchange therapy (PLEX) and Cyclophosphamide (750 mg), with recovery of hemoglobin and platelet levels; however, the patient died due to a complication of the PLEX catheter removal procedure.A total of 18 patients diagnosed with de novo or prevalent Sjögren’s syndrome who had hospital admission with a diagnosis of TMA were included. The mean age was 54.55 years (Standard deviation (SD): 12.45) and 83.33% of the patients corresponded to the female gender.At admission, the mean of hemoglobin was 8.45 g/dL (SD: 2.55) and median platelets of 27250/mm3 (interquartile range (IQR) 10500 - 102000) were found. The most frequent clinical manifestations were central nervous system alterations (50%), followed by bleeding in the skin and renal failure (44.44%) and fever (27.78%). The most frequent antibodies found were anti-Ro (100%), anti-nuclear antibodies (80%) and anti-La (75%). The most frequently prescribed treatment was plasma exchange therapy (83.33%), intravenous steroids (61.11%), oral steroids (61.11%) and cyclophosphamide (27.78%). Of the total patients, 38.89% died and 27.7% had some relapse of TMA.In the group comparison analysis, differences were found in intravenous steroid (81.82% in those who lived vs. 28.57% in those who died p=0.039), use of PLEX (100% in those who survived vs. 57.14% in those who died p = 0.043), fever (9.09% in those who survived and 57.14% in those who died, p = 0.047), admission hemoglobin (7.65 g/dL in those who lived vs. 10.22 g/dL in those who died, p = 0.05), final platelets (148,000 in which who lived and 39,000 in those who died p = 0.02). Then, in the logistic regression analysis, an association was found between mortality and use of intravenous steroids (OR: 0.08, 95% CI 0.009 - 0.83, p = 0.35) and fever at admission (OR: 13.33 95% CI: 1.04 - 169.55, p = 0.046).Conclusion:While the association between TMA and SS is uncommon, so far 18 cases have been reported in the world medical literature. It is typically a condition of women age close to 50 years. The most frequent manifestations are neurological. Among the variables evaluated, only the use of endovenous steroids was associated with a decrease in the probability of mortality; on the contrary, the presence of fever at hospital admission increased the probability of death. The results should be evaluated with caution, since, due to the limited availability of information, they may not be generalizable to clinical practice. More information on this should be obtained in the future.Disclosure of Interests:None declared