A Rare Case of Overlapping Syndrome of ANCA-Associated Glomerulonephritis and Copyright: Systemic Lupus Erythematosus © 2018 Jessica F, Et Al
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www.symbiosisonline.org Symbiosis www.symbiosisonlinepublishing.com Case Report Journal of Rheumatology and Arthritic Diseases Open Access A Rare Case of Overlapping Syndrome of ANCA- Associated Glomerulonephritis and Systemic Lupus Erythematosus Jessica Frey1* and Hajra Shah2 1Neurology Resident, Department of Neurology, West Virginia University, 1 Medical Center Drive, Morgantown WV 26505 USA 2Assistant Professor of Medicine, Department of Rheumatology, West Virginia University, 1 Medical Center Drive, Morgantown WV 26505 USA Received: June 15, 2018; Accepted: July 15, 2018; Published: July 23, 2018 *Corresponding author: Jessica Frey, MD, 99 Lakeside Drive, Morgantown WV 26508, USA. Tel: 412-523-8284; Fax: 304-598-6442; E-mail: [email protected] Abstract mechanisms, prognosis, and management of these two conditions, especially in context of one another. Introduction: Two very interesting rheumatologic diagnoses include Systemic Lupus Erythematosus (SLE) and Anti-Neutrophil Case Report Cytoplasmic Antibody (ANCA)-negative Vasculitis. It is rare to see these two conditions diagnosed in the same patient. This case analyzes We present the case of a 69 year old female with two the complex interactions between these two disease states. simultaneous new diagnoses of SLE and ANCA-negative Vasculitis. Case: This is the case of a 69 year old female who presented with shortness of breath, hematuria, and renal failure. Lab work was pulmonaryHer past medical hypertension. history Herwas presentingsignificant forsymptoms hypothyroidism, included gastro esophageal reflux disease, and recently diagnosed antibody, normal complement levels and a negative Vasculitis panel. one week of increasing shortness of breath, pleuritic chest pain, Renalsignificant biopsy for was elevated consistent ANA (1:1280),with ANCA-associated positive double-stranded Vasculitis (AAV). DNA and joint pain. She was seen at an outside hospital and found Thus the patient met diagnostic criteria for both SLE and AAV and was to have acute kidney injury as well. She was transferred to our treated accordingly. hospital with initial workup revealing of a positive ANA, positive Conclusion: A review of the literature reveals that these typically lupus anticoagulant, and positive anticardiolipin antibody. Vitally separate disease entities when diagnosed together may actually represent a completely new, overlapping syndrome. This case is unique in that there have not been any other cases of a simultaneous on admission, patient had a temperature of 37.2 degrees Celsius, diagnosis of SLE and ANCA-negative Vasculitis without evidence of orientedheart rate to 77, person, respiratory place, andrate time,18, blood and appeared pressure to153/70, be in mildand lupus nephritis reported in the literature. A deeper understanding oxygen saturation 90% on room air. On examination, she was of the mechanism driving these rare diseases can lead to improved prognostication and treatment of these conditions. auscultation, her heart sounded regular in rate and rhythm and herrespiratory lungs had distress. diffuse Hercrackles mucous in her membranes lower left werelung withmoist. good On Systemic lupus erythematosus; ANCA; Vasculitis; Keywords: aeration of her right lung. Her abdomen was soft and non- Autoimmune tender to palpation and bowel sounds were hypoactive. Skin Introduction was warm and dry with no rashes or lesions noted and her extremities had no clubbing, cyanosis, or edema. Continued Systemic Lupus Erythematosus (SLE) and small vessel workup at our hospital revealed that the patient met the criteria vasculitides such as Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis are both uncommon diseases. blood cell casts in urinalysis, thrombocytopenia (values ranged Typically, SLE and ANCA-Associated Vasculitis (AAV) are two for lupus: she had bilateral pleural effusions on chest x-ray, red The renal manifestations of SLE are classically characterized from 43 to 117),and lymphopenia (absolute lymphocyte count bydistinct an immune clinical complexentities, glomerulonephritisand rarely occur simultaneouslywith endocapillary [1]. ranged from 0.24 to 1.37). In addition, laboratory results were significant for a positive double-stranded DNA antibody IgG are characterized by glomerular necrosis and crescent formation towith worsen a value throughout of 551 (more her thanhospital twice stay, normal requiring the referencedialysis. proliferation [2, 3]. In contrast, the renal manifestations of AAV in the absence of cellular proliferation or immune complex Eventuallyvalue), and a positivebiopsy of ANA the kidney(1:1280). was Kidney performed function which continued revealed focal necrotizing glomerulonephritis, red blood cell casts, and be some association between ANCA vasculitides and SLE, but it deposition [4]. There is evidence in the literature that there may following case report, we aim to discuss the pathophysiologic arteriosclerosis with severe intimal fibrosis, suggestive of an is still unclear exactly what this association is [5]. Through the ANCA-associated Vasculitis (Figure 1). There was no evidence Symbiosis Group *Corresponding author email: [email protected] A Rare Case of Overlapping Syndrome of ANCA-Associated Glomerulonephritis and Copyright: Systemic Lupus Erythematosus © 2018 Jessica F, et al Figure 1: Patient’s renal biopsy in a Jones silver stain demonstrating a glomerulus with a focus of fibrinoid necrosis and a red blood cell cast in the oftubule endocapillary next to it, 400 proliferation x magnifications. or immune complex deposition. microscopic Polyangiitis subtype because her biopsy reveals Patient had a negative C-ANCA, indeterminate P-ANCA, and necrotizing Vasculitis of the small vessels without immune In addition, other pertinent negative labs included negative follow up myeloperoxidase and proteinase-3 labs were negative. deposits and no evidence of granulomatous inflammation or A (SSA), Anti-Sjogren’s Syndrome B (SSB), Rheumatoid Factor eosinophils. Our patient also met 6 of the 11 criteria for SLE as (RF),complement and anti-glomerular 3 (C3), complement basement 4 (C4), membrane Anti-Sjogren’s antibody. Syndrome Based (bilateraldefined by pleural the 1997 effusion Update seen of theon CXR),1982 Americanrenal disorder College (red of on this workup, the patient met the criteria for both SLE and AAV. bloodRheumatology cell casts), Revised hematologic Criteria: nonerosivedisorder (lymphopenia arthritis, pleuritis and She was given a course of high dose steroids while in the hospital and subsequently started on cyclophosphamide. Her symptoms phospholipid antibodies), and positive antinuclear antibody. improved and she was suitable for discharge. She continued to thrombocytopenia), immunologic disorder (anti-DNA and anti- be followed by rheumatology and nephrology as an outpatient There are three possibilities to explain the ultimate diagnoses with tri-weekly dialysis sessions. After two months, our patient’s Thus, she fulfilled diagnostic criteria for both AAV and SLE. disease stabilized on azathioprine and cyclophosphamide and happened to meet SLE criteria, she had a separate renal Vasculitis she no longer required dialysis. She remained stable without inof theboth setting AAV andof SLE, SLE or in the this two patient: together she represent had a Vasculitis a single newthat after her diagnosis, she developed pancytopenia and had to be requiring dialysis for another 10 months. However, about a year readmitted to the hospital. Unfortunately, it was a complicated and unique disease entity: an overlapping syndrome. hospital course involving neutropenic fever which did not the question of the underlying mechanisms driving these two improve with antibiotics and pancytopenia which did not improve diseases.This caseSince is there significant are many for manyfeatures reasons. of SLE First,and AAVit raises that with multiple transfusions of blood products. She additionally overlap, it is possible that they are triggered together by similar had severe metabolic acidosis thought to be secondary to splenic infarctions and required intubation and pressor support. Family ultimately decided to pursue a comfort measures approach. Thus triggeredmechanisms. the ANCAIndeed, associated one study Vasculitis found tothat develop in 9% into of rapidlycases, the patient was compassionately extubated and she passed away there was some underlying chronic inflammatory process that shortly thereafter. underlying the renal manifestations of each of these diseases areproliferating complex. Althoughglomerulonephritis classic lupus [7]. nephritis The path is thoughtphysiologies to be Discussion secondary to immune complex deposition, it has been suggested in the literature that class IV lupus may be caused by a different this patient’s Vasculitis diagnosis is most consistent with the Based on the Chapel Hill consensus conference in 2012, mechanism [3]. Class IV lupus nephritis often exhibits prominent Citation: Page of 4 Jessica F, Hajra S (2018) A Rare Case of Overlapping Syndrome of ANCA-Associated Glomerulonephritis and Systemic 2 Lupus Erythematosus. J Rheumatol Arthritic Dis 3(2): 1-4. A Rare Case of Overlapping Syndrome of ANCA-Associated Glomerulonephritis and Copyright: Systemic Lupus Erythematosus © 2018 Jessica F, et al glomerular necrosis and little or no immune complex deposition. in the literature, there have also been cases of ANCA positive Some studies have suggested that crescentic necrotizing pauci- glomerulonephritis in the setting of SLE as well