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Case Report Protein-Losing Enteropathy and Iga Nephropathy in a Man with Systemic Lupus Erythematosus: a Case Report

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Case Report Protein-Losing Enteropathy and Iga Nephropathy in a Man with Systemic Lupus Erythematosus: a Case Report Int J Clin Exp Med 2018;11(12):13945-13948 www.ijcem.com /ISSN:1940-5901/IJCEM0072570 Case Report Protein-losing enteropathy and IgA nephropathy in a man with systemic lupus erythematosus: a case report Xiaochang Xu, Yimin Zhang Division of Nephrology, The Sixth Affiliated Hospital, Sun Yat-sen University, Guangzhou, China Received January 11, 2018; Accepted September 10, 2018; Epub December 15, 2018; Published December 30, 2018 Abstract: Protein losing enteropathy (PLE) is a rare gastrointestinal manifestation of systemic lupus erythematosus (SLE). The causes of non-lupus nephropathies associated with SLE were rarely described. Here we presented a case of oedema with ascites from serve hypoalbuminaemia secondary to lupus-associated PLE. Renal biopsy revealed IgA deposits in isolated mesangium, consistent with the pathological feature of primary IgA nephropathy (IgAN). Moreover, a full clinical remission was achieved with a course of intravenous cyclophosphamide therapy. Keywords: Systemic lupus erythematosus (SLE), protein-losing enteropathy (PLE), IgA nephropathy (IgAN), CA125 Introduction ulcer, and received angiotensin receptor block- ers (ARB) instead. In his visit to the clinic of Protein-losing enteropathy (PLE) is generally another institution on February 2017, he com- diagnosed in patients with hypoproteinaemia plained gradual abdominal distension, general- after other causes, such as malnutrition, pro- ized swelling, and a 7.0-kg weight gain. There teinuria and impaired protein synthesis due to was no arthralgia, oral ulcer, butterfly malar cirrhosis are excluded. It is a rare condition rash or alopecia. Laboratory investigations characterized by a loss of serum protein into revealed a normal full blood count and creati- the gastrointestinal tract resulting in hypopro- nine level, and the urinary protein was 0.6 g/24 teinaemia, which manifests as oedema, asci- h. He was found hypoalbuminaemic with serum tes, malnutrition, and pleural and pericardial albumin of 12 g/L (normal range 35-50 g/L). effusions. Primary IgA nephropathy (IgAN), the His serology profile demonstrated positive for most frequent primary glomerulopathy in the antinuclear antibody (ANA) and hypocomple- world population, was rarely described in asso- mentemia. The CA125 level was markedly ciation with systemic lupus erythematosus raised to 2929 IU/mL (normal range 0-30 IU/ (SLE). In the present study, we reported a case mL). Ascetic fluid assessment showed a low of SLE-associated PLE and IgAN with severe fluid protein, suggesting a transudate. Cytology hypoalbminaemia secondary to lupus in a man. tests found suspected low-differentiated ade- nocarcinoma cells on two separate occasions. Case report The abdominal CT scan with contrast and PET- CT scan demonstrated no tumor but polyserosi- A 46-year-old Chinese male was admitted in tis. Electronic gastroscopy showed healing March 2017 with a three-month history of pro- ulcers. According to the American College of gressive anasarca. Three months ago, the Rheumatology’s revised criteria in 1997, the patient received a diagnosis of IgAN based on patient did not meet the diagnosis of SLE the pathology report of mesangial hypercellu- because only positive ANA, polyserositis, and a larity with isolated IgA deposits in his renal biopsy-proven IgAN were detected. Since biopsy sample. The patient did not respond to a Malignant tumor cannot be ruled out, the month of corticosteroid therapy. Two months patient received low dose prednisone treat- before the present admission, he defaulted ment and was admitted into our hospital in corticosteroid treatment because of gastric March 2017. Protein-losing enteropathy and IgAN in SLE On admission, he complained of serious ab- 4-22% of all SLE patients and are misdiag- dominal distension and intermittent diarrhea nosed easily for special clinical manifesta- for three years. His anti-ds-DNA level was with- tion and serological results [1]. Studies have in the normal range, and anti-U1RNP and anti- shown that serositis is a more prominent clini- Smith antigen were positive. A test for ANA was cal manifestation and auto-antibody positive positive at a dilution of 1:1000, with a speckled rate is lower in male compared with female SLE pattern. Complements (C3 and C4) were low at patients [1, 2]. It is known that the usage of the 0.62 g/L (normal range 0.8-1.6 g/L) and 0.07 new SLICC classification criteria relative to the g/L (normal range 0.16-0.38 g/L), respectively. current ACR classification criteria resulted in Cytomegalovirus, hepatitis B and C were nega- fewer misclassifications (49 versus 70) and led tive in serological tests. The CA125 levels were to greater sensitivity (94% versus 86%) [3]. We markedly raised at 3575 IU/mL (normal range have performed well in the case, i.e. the male 0-30 IU/mL). A serum albumin was 12.45 g/L SLE patient with ascites, positive ANA and (normal range 35-50 g/L). Values for 24-hour hypocomplementemia, using the new classifi- urine-protein excretion were within 0.5 g. Given cation criteria. the concern of a malignant tumor, the patient underwent a Laparoscopic exploration, in which PLE was manifested as a severe hypoalbumin- a normal enterocoelia without any intra-abdom- emia due to plasma protein leaking into gastro- inal tumor deposits nor evidence of infection intestinal tract. Causes of PLE include erosive were shown. gastrointestinal lesions (sarcoidosis, ulcera- tive colitis, pseudo membranous enteritis, gas- During the admission in April 2017, his diagno- trointestinal lymphoma, peptic ulcer), non-ero- sis was changed to probable systemic lupus sive gastrointestinal lesions (celiac disease, erythematosus (SLE) based on the presence microscopically colitis, rheumatic disease), and of polyserositis, hypocomplementaemia, high disorders involving increased central venous titre ANA and positive anti-Smith antigen, pressure or mesenteric lymphatic obstruction according to the SLICC Revision of the ACR (intestinal lymphatic vessels dilated disease, Classification Criteria in 2009. A diagnosis of congestive heart failure, portal hypertension, lupus associated protein-losing enteropathy after intestinal lymphatic fistula and peritoneal (LUPLE) was made after other causes of hypo- sclerosis) [4]. albuminaemia, such as malnutrition, protein- uria and impaired protein synthesis due to cir- SLE is a rare cause of PLE, the possible mecha- rhosis were ruled out. A Technetium 99m- nism underlying which may be associated with labelled (99mTc) human serum albumin (HSA) intestinal mucosa or vascular injury and in- scintigraphy or faecal alpha-1-antityrpsin clear- creased capillary permeability of intestinal ance (FAAC) test, which would have confirmed mucosa mediated by cytokines or complement the diagnosis, was not available at our hospital. as well as intestinal lymphangiectasia becau- The patient received a seven-day course of se of extrusion of oedematous interstitial [5]. intravenous methylprednisolone (80 mg daily), Some patients also have hypoglobulinemia followed by a course of intravenous cyclophos- because immunoglobulin can also leak into phamide weekly at 1 g per week for two weeks the intestinal lumen [6]. 99mTc-labelled HSA and 50 mg of oral prednisolone daily at approxi- scan Radiolabelled albumin and faecal clear- mately 1 mg/kg per day. The planned second ance of alpha 1-antitrypsin (a1-AT) are classical dose of cyclophosphamide was completed in approaches that have been used for the diag- May 2017. He did well during the following 2 nosis of protein malabsorption and intestinal months but complained about painful knee losses [7, 8]. However, both approaches were arthritis on his last review in June 2017. His not available in our hospital. The patient was ascites were minimal, serum albumin was 41 diagnosed with SLE because of hypoalbumin- g/L, ANA remained positive in low titer, and emia. However, inadequate intake, proteinuria, CA125 has been tapered to a low level. liver dysfunction or thyroid dysfunction were not found in the patient. Therefore, PLE should Discussion be taken into account. SLE, a multi-organ, autoimmune disease, main- CA-125, recognized by murine monoclonal anti- ly affects women. The peak age at onset ranges body OC125, has been proposed as a mark- from 20-40 years. Males with SLE represent er for carcinoma. However, CA-125 elevation 13946 Int J Clin Exp Med 2018;11(12):13945-13948 Protein-losing enteropathy and IgAN in SLE was observed in many physiologic and pa- brane [18, 21]. In healthy adults, the mucosal thologic conditions including pregnancy, dur- immune system accounts for about 80% of the ing menses, and ascites [9, 10]. It can be immune system, and the secreted IgA (sIgA) of detected immunohistochemically in the meso- intestinal mucosal played a primary role in thelial cells of the peritoneum, pleura, and peri- humoral immunity [18, 22]. Various studies cardium [9]. imply that dysregulated intestinal mucosal immunity caused by dietary components, intes- Serositis, a classification criterion for SLE, tinal microbiota, and intestinal diseases pro- can be present in the form of peritonitis, pleuri- mote the production of sIgA. SIgA is then tis, and pericarditis with accompanying fluid deposited in the glomerular mesangium, even- accumulation, all of which may result in the tually leading the development of IgAN [23]. elevation of CA-125. It was reported that CA125 It is reasonable to postulate that the tonsillec- was detected in 15% of SLE patients in a tomy, a simple way of removing part of patho-
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