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Saudi Journal of Kidney Diseases and Transplantation
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Table of Contents   
CASE REPORT  
Year : 2018  |  Volume : 29  |  Issue : 2  |  Page : 429-434
Acute gastric dilatation in a patient with lupus nephritis: An uncommon presentation of lupus mesenteric vasculitis


1 Department of Nephrology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India
2 Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry, India

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Date of Web Publication10-Apr-2018
 

   Abstract 

Abdominal symptoms are common in patients with lupus nephritis and are often attributed to drugs or uremia per se. Lupus mesenteric vasculitis (LMV) or lupus enteritis is a rare entity reported in patients with active systemic lupus erythematosus. It usually occurs in patients with a long-standing history of lupus with high disease activity. Usually, small bowel is predominantly affected. The stomach and rectum are spared in view of significant collateral circulation. Here, we describe an 18-year-old boy who presented with nephrotic syndrome without any extrarenal features of lupus. On subsequent evaluation, he was found to have active lupus nephritis. He developed acute gastric dilatation secondary to extensive LMV. Imaging showed an extensive involvement of gastrointestinal tract from the stomach to the sigmoid colon, sparing the rectum. To the best of our knowledge, this is the first report of LMV presenting as acute gastric dilatation.

How to cite this article:
Ezhilnilavan S, Priyamvada P S, Haridasan S, Rajesh N G, Parameswaran S. Acute gastric dilatation in a patient with lupus nephritis: An uncommon presentation of lupus mesenteric vasculitis. Saudi J Kidney Dis Transpl 2018;29:429-34

How to cite this URL:
Ezhilnilavan S, Priyamvada P S, Haridasan S, Rajesh N G, Parameswaran S. Acute gastric dilatation in a patient with lupus nephritis: An uncommon presentation of lupus mesenteric vasculitis. Saudi J Kidney Dis Transpl [serial online] 2018 [cited 2022 May 17];29:429-34. Available from: https://www.sjkdt.org/text.asp?2018/29/2/429/229279

   Introduction Top


Lupus mesenteric vasculitis (LMV) or lupus enteritis is an under-recognized entity described in patients with active systemic lupus erythematosus (SLE). The clinical picture of LMV is often nonspecific, which may vary from mild to severe abdominal pain, diarrhea, and vomiting. A high index of clinical suspicion is needed for diagnosing LMV. The disease often shows an excellent response to corticosteroid therapy but can rapidly progress to bowel gangrene if prompt treatment is not instituted. The diagnosis can be made from a suggestive radiological picture in contrast-enhanced computerized tomography (CT) or by tissue biopsy.[1],[2] Here, we report a patient who presented with nephrotic syndrome secondary to lupus nephritis developing acute onset massive dilatation of the stomach and small bowel with imaging showing characteristic radiological features of LMV.


   Case Report Top


An 18-year-old mentally challenged boy, born by nonconsanguineous marriage, was admitted with gradually progressive edema of six months and reduced urine output for 20-day duration. There was no history of fever, arthritis, rashes, photosensitivity, alopecia, or Raynaud phenomenon. At the time of admission, he was not having any abdominal symptoms. On physical examination, he had features suggestive of Marfan syndrome. He was having generalized edema, ascites, and bilateral pleural effusions. His blood pressure was 150/90 mm Hg, and jugular venous pressure was not elevated. The cardiovascular system examination was unremarkable except for a flow murmur in pulmonary area. There was no hepatosplenomegaly, and bowel sounds were normally present. His serum creatinine was 1.7 mg/dL at admission. Urine routine showed 3+ proteinuria and plenty of dysmorphic red blood cells (RBCs), RBC casts and a few white blood cells. His hemoglobin (Hb) was 5.3 g/dL; peripheral smear showed normocytic normochromic anemia. An initial workup for hemolysis was noncontributory. The other relevant investigations at the time of admission are given in [Table 1]. The patient was started on parenteral loop diuretics and other supportive measures. The patient's urine output gradually decreased in spite of optimal diuretic therapy. The renal failure was attributed to acute tubular necrosis secondary to nephrotic syndrome. In view of progressive renal dysfunction, he was started on hemodialysis with a venous catheter in the right internal jugular vein.
Table 1: Blood investigations.

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The patient's Hb levels failed to improve in spite of receiving four units of packed cell transfusions. Hence, a repeat hemolytic evaluation was done. A peripheral smear showed occasional spherocytes. The reticulocyte proliferation index was 3, and a repeat Coombs test was strongly positive (1:640 dilution). Further investigations revealed low complement component C3 and C4 levels, high titer antinuclear antibody and double-stranded DNA positivity. Meanwhile, the patient developed progressive abdominal distension with melena. A plain X-ray abdomen erect view revealed acute dilatation of the stomach and the small bowel without any significant air-fluid levels [Figure 1]. The patient was kept nil per oral, and the stomach was decompressed with Ryle's tube aspiration. The abdominal sonography revealed a moderate degree of ascites, dilated stomach and intestine loops without peristalsis. Contrast-enhanced CT scan revealed diffuse swelling of the stomach, small and large bowel walls with intestinal dilatation with “target sign” and attenuation of mesenteric fat and engorgement of mesenteric vessels producing the classical “comb sign” characteristic of LMV [Figure 2].
Figure 1: X-ray abdomen erect anteroposterior view showing massive dilatation of the stomach extending up to the level of L4 vertebra and dilated jejunal loops.

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Figure 2: (a) CECT abdomen showing significant submucosal edema of the stomach with rugosities; Ryle's tube in situ, (b) small bowel mucosal edema with wall thickening with submucosal contrast enhancement (target sign), (c) CECT abdomen showing increased attenuation of mesenteric fat, and (d) CECT abdomen showing prominent engorgement of the mesenteric vessels with palisade or comb-like arrangement (comb sign).
CECT: Contrast-enhanced computerized tomography.


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The patient was given injection methylprednisolone 1 g pulses once daily for three days followed by injection dexamethasone 4 mg three times a day for next four days, followed by oral prednisolone at dose of 1 mg/KBW. Intravenous immunoglobulin was added as an adjunctive therapy considering severe complications and high SLE Disease Activity Index (SLEDAI index = 46). His abdominal distention settled, and gradually the patient was started on oral feeds. His Hb levels also stabilized. The patient continued to remain dialysis dependent. A kidney biopsy showed Class 4 lupus nephritis [Figure 3] and [Figure 4]. Seven days after receiving corticosteroids, the patient developed fever and cough. Chest X-ray showed left lower lobe pneumonia and pleural effusions. His total blood white blood counts at the time of developing sepsis were 7080 with 94% polymorphs. His serum total proteins and albumin were 4.3 g/dL and 1.0 g/dL, respectively. Leukopenia or low immunoglobulin levels were not documented during the hospital stay. Blood culture grew Pseudomonas aeruginosa species. In spite of antibiotics and supportive management, the patient succumbed to sepsis.
Figure 3: (a) Kidney biopsy showing glomerulus with global mesangial proliferation and wire loop lesions suggestive of lupus nephritis (H and E, ×40), (b) Kidney biopsy showing glomerulus with mesangial and endocapillary proliferation with segmental cellular crescent and hyaline thrombi. (PAS, ×40): Arrowheads showing crescents, hyaline thrombi, and wire loops.

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Figure 4: IgG: Section shows 3+ linear and granular deposits of IgG along glomerular capillary basement membrane, FITC stain, ×400 IgM: Section shows 2+ deposits of IgM in the mesangial region, FITC stain, ×400 C1q :Section shows 2+ deposits of C1q along the glomerular capillary basement membrane and Bowman's capsule, FITC stain, ×400.

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   Discussion Top


Abdominal symptoms are common in patients with SLE. They often result from adverse effects of medications or infections. In a patient with lupus nephritis and severe renal dysfunction, the uremic milieu per se might contribute to the abdominal symptoms. Gut involvement arising from lupus activity is much more uncommon compared to the involvement of visceral organs such as kidney. LMV is often under recognized owing to its non-specific clinical symptoms and rarity of presentation. The reported prevalence varies from 0.9% to 9.7%, with a comparatively higher prevalence reported from Asian countries.[1] It almost exclusively occurs in patients with high lupus activity scores.[1],[3],[4] It is reported that patients with high titers of antiendothelial antibodies and antiphospholipid antibodies are more vulnerable for LMV.[3],[4] An under diagnosis of LMV is common; it is estimated that the prevalence might be as high as 29% to 65% of lupus patients who presents with acute abdominal pain.[5] Even though LMV occurs later in the course of the disease, it can rarely present as the first clinical manifestation of lupus as well.[6]

The symptoms of LMV vary from high intensity, diffuse abdominal pain (70%–100%) with bloating and anorexia, diarrhea (30%–60%), and vomiting (60%–80%).[5],[7] LMV occurs as a result of immune complex deposition and complement activation resulting in leukocytoclastic vasculitis as well as thrombosis of small mesenteric vessels leading to submucosal edema of intestine and ischemia. Even though LMV is a vasculitic event, histological confirmation is not necessary for diagnosis. Endoscopy-guided biopsy is often noncontributory as the characteristic histological signs of vasculitis are present in vessels, which is often inaccessible by biopsy. Hence, contrast-enhanced CT scanning has become the gold standard for diagnosis of LMV. Typical radiologic features include the following: (a) thickened bowel wall >3 mm with enhancing outer (muscularis propria/serosa) and inner (mucosa) layers with a hypoenhancing middle layer resulting from submucosal edema (target sign) with dilated bowel loops, (b) engorgement of mesenteric vessels with increased number of visible vessels (comb sign), and (c) increased attenuation of mesenteric fat and ascites. Bowel wall involvement is mostly multisegmental and not confined to a single vascular territory. The jejunum and ileum are the most commonly involved sites. Isolated vasculitic involvement of the stomach and the rectum are rare because of collateral circulation.[8]

Lupus enteritis is reversible and steroid responsive. For corticosteroid-resistant, gastrointestinal (GI) vasculitis, successful treatment with cyclophosphamide/rituximab can be achieved.[9] If undiagnosed, the disease can rapidly progress to bowel gangrene. Even though our patient showed a favorable response to immunosuppression, he developed sepsis with pneumonia, probably secondary to catheter-related bacteremia, and could not be salvaged in spite of antibiotic therapy.

Our patient had a few atypical features. LMV occurred fairly early in the course of lupus. In fact, lupus was not initially suspected as there were no systemic features of lupus at the time of admission. Initial workup for hemolysis was noncontributory, and the anemia was attributed to iron loses resulting from the nephrotic state. The subsequent hemolysis which developed after admission alerted us toward the possibility of an immunological event. Another interesting aspect is the initial presentation as acute gastric dilatation. The acute gastric dilatation was initially attributed to the metabolic and electrolyte disturbances secondary to the severe kidney injury. Generalized hollow visceral dilatation of the GI tract and biliary and urinary tracts is a well-described entity in lupus, but rather than the stomach, the small and large bowel is predominantly involved. This is attributed to immune-mediated smooth muscle myopathy, but the typical CT signs of mesenteric vasculitis are absent.[10],[11] To the best of our knowledge, LMV presenting as acute gastric dilatation has not described so far. The patient showed radiological evidence of gut wall involvement extending from the stomach to the sigmoid colon. The radiological features of stomach wall involvement in the form of extensive sub-mucosal edema are suggestive of a direct vasculitic process, rather than functional dilation secondary to enteritis or metabolic disturbances. Such extensive involvement of the GI tract has rarely been described in LMV.


   Conclusions Top


LMV is an uncommon entity which can complicate the clinical course of lupus. LMV can occur in previously undiagnosed cases of lupus and early in the course of the disease as well. A variety factors including drugs, metabolic factors, and abdominal pathologies such as pancreatitis can contribute to abdominal symptoms in lupus. Since the presentation is often nonspecific, LMV can be easily overlooked. In acutely uremic patients having lupus nephritis, the abdominal symptoms are often attributed to the altered metabolic milieu. Physicians treating lupus nephritis need to be aware of this rare complication. LMV needs to be considered in the differential diagnosis in patients with lupus presenting with acute abdominal symptoms.

Conflict of interest: None declared.

 
   References Top

1.
Tian XP, Zhang X. Gastrointestinal involvement in systemic lupus erythematosus: Insight into pathogenesis, diagnosis and treatment. World J Gastroenterol 2010;16:2971-7.  Back to cited text no. 1
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2.
Janssens P, Arnaud L, Galicier L, et al. Lupus enteritis: From clinical findings to therapeutic management. Orphanet J Rare Dis 2013;8:67.  Back to cited text no. 2
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3.
Kwok SK, Seo SH, Ju JH, et al. Lupus enteritis: Clinical characteristics, risk factor for relapse and association with anti-endothelial cell antibody. Lupus 2007;16:803-9.  Back to cited text no. 3
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4.
Cervera R, Espinosa G, Cordero A, et al. Intestinal involvement secondary to the antiphospholipid syndrome (APS): Clinical and immunologic characteristics of 97 patients: Comparison of classic and catastrophic APS. Semin Arthritis Rheum 2007;36:287-96.  Back to cited text no. 4
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5.
Ju JH, Min JK, Jung CK, et al. Lupus mesenteric vasculitis can cause acute abdominal pain in patients with SLE. Nat Rev Rheumatol 2009;5:273-81.  Back to cited text no. 5
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6.
Sran S, Sran M, Patel N, Anand P. Lupus enteritis as an initial presentation of systemic lupus erythematosus. Case Rep Gastrointest Med 2014;2014:3.  Back to cited text no. 6
    
7.
Lee CK, Ahn MS, Lee EY, et al. Acute abdominal pain in systemic lupus erythema- tosus: Focus on lupus enteritis (gastrointestinal vasculitis). Ann Rheum Dis 2002;61:547-50.  Back to cited text no. 7
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8.
Byun JY, Ha HK, Yu SY, et al. CT features of systemic lupus erythematosus in patients with acute abdominal pain: Emphasis on ischemic bowel disease. Radiology 1999;211:203-9.  Back to cited text no. 8
    
9.
Waite L, Morrison E. Severe gastrointestinal involvement in systemic lupus erythematosus treated with rituximab and cyclophosphamide (B-cell depletion therapy). Lupus 2007;16:841- 2.  Back to cited text no. 9
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10.
Mok MY, Wong RW, Lau CS. Intestinal pseudo-obstruction in systemic lupus erythe-matosus: An uncommon but important clinical manifestation. Lupus 2000;9:11-8.  Back to cited text no. 10
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11.
Hill PA, Dwyer KM, Power DA. Chronic intestinal pseudo-obstruction in systemic lupus erythematosus due to intestinal smooth muscle myopathy. Lupus 2000;9:458-63.  Back to cited text no. 11
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Correspondence Address:
Dr. P S Priyamvada
Department of Nephrology, Jawaharlal Institute of Postgraduate Medical Education and Research, Puducherry - 605 006
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1319-2442.229279

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