Letters
85
Abdominal pain in systemic lupus erythematosus: lupus enteritis, mesenteric thrombosis, or median arcuate ligament syndrome? N Kamalaraj1,2, T Tsai1, D Massasso1 Rheumatology Department, Liverpool Hospital, Sydney, and 2University of New South Wales, Australia
Scand J Rheumatol Downloaded from informahealthcare.com by Selcuk Universitesi on 01/11/15 For personal use only.
1
Abdominal pain in patients with systemic lupus erythematosus (SLE) can be a challenging diagnostic dilemma. Lupus-specific aetiologies such as lupus enteritis, mesenteric vasculitis, or thrombosis are difficult to differentiate clinically. The emerging syndrome of median arcuate ligament compression is another potential mimic that may result in inappropriate vascular intervention or anticoagulation in young lupus patients. A 38-year-old woman with recently diagnosed SLE and antiphospholipid (aPL) antibody-negative popliteal artery thrombosis, on warfarin, presented with a 3-week history of postprandial abdominal pain, diarrhoea, weight loss, and active systemic lupus features, including alopecia and facial rash. Initial examination showed the patient was afebrile, in sinus tachycardia (120 beats/min), and normotensive. Her abdomen was diffusely tender with voluntary guarding but no abdominal bruits. Peripheral pulses were normal. The erythrocyte sedimentation rate (ESR) was 125 mm/h, C-reactive protein (CRP) 3 mg/L, with full blood count, biochemistry and liver function tests normal. The international normalized ratio (INR) was subtherapeutic at 0.9. Antinuclear antibodies (ANA) were > 1:1280, double-stranded DNA (dsDNA) 235 IU/ mL (ULN ¼ 99), with C3 0.51 g/L (LLN ¼ 0.79) and C4 0.09 g/L (LLN ¼ 0.16). Repeat aPL were negative. Stool cultures and urine tests for casts, protein, and culture were unremarkable. A computed tomography (CT) mesenteric angiogram demonstrated a new short segment stenosis of the proximal coeliac trunk with associated post-stenotic dilatation (Figure 1B) compared to her CT angiogram 5 weeks earlier when she presented with popliteal artery thrombosis (Figure 1A). Therapeutic enoxaparin for possible mesenteric thrombosis was commenced. However, comparison of her two CT angiograms revealed differing phases of respiration, and the differential diagnosis of median arcuate ligament syndrome (MALS) was raised. Visceral Doppler ultrasound showed normal flow and velocity in the intra-abdominal arteries, thereby excluding coeliac artery thrombosis and MALS. The CT findings were instead due to physiological coeliac artery compression by the median arcuate ligament. Because of her recent CT radiation exposure, magnetic resonance enterography (MRE) was performed showing bowel wall thickening and oedema consistent with
Figure 1. (A) Computed tomography (CT) mesenteric angiogram obtained in January 2013 showing the normal coeliac artery. Study performed during inspiration. (B) CT mesenteric angiogram obtained in March 2013 showing focal hook-shaped stenosis of the proximal coeliac trunk with distal dilatation. Study performed during expiration.
terminal ileitis (Figure 2), supporting a diagnosis of lupus enteritis. Upper and lower endoscopies with random biopsies, 1 week after treatment, were normal. The patient was given bowel rest, intravenous methylprednisolone 500 mg daily for 3 days, and subsequent oral prednisone (40 mg daily). Her gastrointestinal symptoms resolved rapidly, and azathioprine was commenced. She continues to improve at her 16-month follow-up on azathioprine and low-dose prednisone. This case highlights the potential misdiagnoses of coeliac artery thrombosis or MALS on CT angiography, especially in patients with inflammatory rheumatic disease who have high thrombotic risk regardless of aPL antibody status (1–5). MALS is a controversial diagnosis in patients with an aberrant low median arcuate ligament that passes anterior to the coeliac trunk, causing arterial obstruction. Respiration-dependent epigastric bruit is sometimes present. As in our case, MALS occurs typically in young (20–40-year-old), female patients, and manifests with
www.scandjrheumatol.dk
86
Letters
similar sensitivity (8). Given that early or mild cases of lupus enteritis may be normal on conventional CT and/or endoscopy (9, 10), MRE may be an emerging diagnostic tool in such cases. This case illustrates the potential diagnostic pitfalls of apparent coeliac artery stenosis on CT angiography, which may be misinterpreted as coeliac trunk thrombosis or MALS. Accurate diagnosis with visceral Doppler studies helps to avoid unnecessary and potentially harmful vascular intervention or anticoagulation. Furthermore, MRE may be a useful emerging diagnostic tool with no radiation risk for difficult cases of young lupus enteritis.
Scand J Rheumatol Downloaded from informahealthcare.com by Selcuk Universitesi on 01/11/15 For personal use only.
References
Figure 2. Magnetic resonance enterography (MRE) demonstrating focal thickening of the terminal ileum.
postprandial epigastric pain, diarrhoea, and weight loss (6). CT angiography shows a characteristic hook-shaped stenosis of the proximal coeliac trunk, evident in expiration (6). As 10–24% of healthy people may have the anatomical median arcuate ligament variant without true vascular obstruction (6), MALS can be misdiagnosed in patients with abdominal pain who undergo CT abdominal angiography to exclude vasculitis or thrombosis. Visceral Doppler is suggestive of MALS if the peak expiratory systolic velocity is > 200 cm/s, or the velocity is > 3:1 ratio compared to the adjacent aortic flow (7). The normal visceral arterial Doppler in this case was crucial in differentiating the respiration-related, physiological coeliac artery compression from coeliac artery thrombosis or MALS. The diagnosis of lupus enteritis in our case was corroborated by MRE. This was preferred over conventional CT enterography because of our patient’s young age and recent radiation exposure. MRE is currently used in assessing Crohn’s disease, particularly in younger patients, as a radiation-sparing alternative to CT enterography with
www.scandjrheumatol.dk
1. Zoller B, Li X, Sundquist J, Sundquist K. Risk of pulmonary embolism in patients with autoimmune disorders: a nationwide follow-up study from Sweden. Lancet 2012;379:244–9. 2. Zoller B, Li X, Sundquist J, Sundquist K. Risk of subsequent coronary heart disease in patients hospitalized for immunemediated diseases: a nationwide follow-up study from Sweden. PLoS One 2012;7:e33442. 3. Tektonidou MG, Laskari K, Panagiotakos DB, Moutsopoulos HM. Risk factors for thrombosis and primary thrombosis prevention in patients with systemic lupus erythematosus with or without antiphospholipid antibodies. Arthritis Rheum 2009;61:29–36. 4. Al-Homood IA. Thrombosis in systemic lupus erythematosus: a review article. ISRN Rheumatol 2012;2012:428269. 5. D’Cruz DP, Khamashta MA, Hughes GR. Systemic lupus erythematosus. Lancet 2007;369:587–96. 6. Horton KM, Talamini MA, Fishman EK. Median arcuate ligament syndrome: evaluation with CT angiography. Radiographics 2005;25:1177–82. 7. Ozel A, Toksoy G, Ozdogan O, Mahmutoglu AS, Karpat Z. Ultrasonographic diagnosis of median arcuate ligament syndrome: a report of two cases. Med Ultrason 2012;14:154–7. 8. Grand DJ, Beland M, Harris A. Magnetic resonance enterography. Radiol Clin North Am 2013;51:99–112. 9. Smith LW, Petri M. Lupus enteritis: an uncommon manifestation of systemic lupus erythematosus. J Clin Rheumatol 2013;19:84–6. 10. Tian XP, Zhang X. Gastrointestinal involvement in systemic lupus erythematosus: insight into pathogenesis, diagnosis and treatment. World J Gastroenterol 2010;16:2971–7.
Narainraj Kamalaraj, 60 Mulgray Avenue, Baulkham Hills, NSW 2153, Australia. E-mail: [email protected] Accepted 11 September 2014
85
Abdominal pain in systemic lupus erythematosus: lupus enteritis, mesenteric thrombosis, or median arcuate ligament syndrome? N Kamalaraj1,2, T Tsai1, D Massasso1 Rheumatology Department, Liverpool Hospital, Sydney, and 2University of New South Wales, Australia
Scand J Rheumatol Downloaded from informahealthcare.com by Selcuk Universitesi on 01/11/15 For personal use only.
1
Abdominal pain in patients with systemic lupus erythematosus (SLE) can be a challenging diagnostic dilemma. Lupus-specific aetiologies such as lupus enteritis, mesenteric vasculitis, or thrombosis are difficult to differentiate clinically. The emerging syndrome of median arcuate ligament compression is another potential mimic that may result in inappropriate vascular intervention or anticoagulation in young lupus patients. A 38-year-old woman with recently diagnosed SLE and antiphospholipid (aPL) antibody-negative popliteal artery thrombosis, on warfarin, presented with a 3-week history of postprandial abdominal pain, diarrhoea, weight loss, and active systemic lupus features, including alopecia and facial rash. Initial examination showed the patient was afebrile, in sinus tachycardia (120 beats/min), and normotensive. Her abdomen was diffusely tender with voluntary guarding but no abdominal bruits. Peripheral pulses were normal. The erythrocyte sedimentation rate (ESR) was 125 mm/h, C-reactive protein (CRP) 3 mg/L, with full blood count, biochemistry and liver function tests normal. The international normalized ratio (INR) was subtherapeutic at 0.9. Antinuclear antibodies (ANA) were > 1:1280, double-stranded DNA (dsDNA) 235 IU/ mL (ULN ¼ 99), with C3 0.51 g/L (LLN ¼ 0.79) and C4 0.09 g/L (LLN ¼ 0.16). Repeat aPL were negative. Stool cultures and urine tests for casts, protein, and culture were unremarkable. A computed tomography (CT) mesenteric angiogram demonstrated a new short segment stenosis of the proximal coeliac trunk with associated post-stenotic dilatation (Figure 1B) compared to her CT angiogram 5 weeks earlier when she presented with popliteal artery thrombosis (Figure 1A). Therapeutic enoxaparin for possible mesenteric thrombosis was commenced. However, comparison of her two CT angiograms revealed differing phases of respiration, and the differential diagnosis of median arcuate ligament syndrome (MALS) was raised. Visceral Doppler ultrasound showed normal flow and velocity in the intra-abdominal arteries, thereby excluding coeliac artery thrombosis and MALS. The CT findings were instead due to physiological coeliac artery compression by the median arcuate ligament. Because of her recent CT radiation exposure, magnetic resonance enterography (MRE) was performed showing bowel wall thickening and oedema consistent with
Figure 1. (A) Computed tomography (CT) mesenteric angiogram obtained in January 2013 showing the normal coeliac artery. Study performed during inspiration. (B) CT mesenteric angiogram obtained in March 2013 showing focal hook-shaped stenosis of the proximal coeliac trunk with distal dilatation. Study performed during expiration.
terminal ileitis (Figure 2), supporting a diagnosis of lupus enteritis. Upper and lower endoscopies with random biopsies, 1 week after treatment, were normal. The patient was given bowel rest, intravenous methylprednisolone 500 mg daily for 3 days, and subsequent oral prednisone (40 mg daily). Her gastrointestinal symptoms resolved rapidly, and azathioprine was commenced. She continues to improve at her 16-month follow-up on azathioprine and low-dose prednisone. This case highlights the potential misdiagnoses of coeliac artery thrombosis or MALS on CT angiography, especially in patients with inflammatory rheumatic disease who have high thrombotic risk regardless of aPL antibody status (1–5). MALS is a controversial diagnosis in patients with an aberrant low median arcuate ligament that passes anterior to the coeliac trunk, causing arterial obstruction. Respiration-dependent epigastric bruit is sometimes present. As in our case, MALS occurs typically in young (20–40-year-old), female patients, and manifests with
www.scandjrheumatol.dk
86
Letters
similar sensitivity (8). Given that early or mild cases of lupus enteritis may be normal on conventional CT and/or endoscopy (9, 10), MRE may be an emerging diagnostic tool in such cases. This case illustrates the potential diagnostic pitfalls of apparent coeliac artery stenosis on CT angiography, which may be misinterpreted as coeliac trunk thrombosis or MALS. Accurate diagnosis with visceral Doppler studies helps to avoid unnecessary and potentially harmful vascular intervention or anticoagulation. Furthermore, MRE may be a useful emerging diagnostic tool with no radiation risk for difficult cases of young lupus enteritis.
Scand J Rheumatol Downloaded from informahealthcare.com by Selcuk Universitesi on 01/11/15 For personal use only.
References
Figure 2. Magnetic resonance enterography (MRE) demonstrating focal thickening of the terminal ileum.
postprandial epigastric pain, diarrhoea, and weight loss (6). CT angiography shows a characteristic hook-shaped stenosis of the proximal coeliac trunk, evident in expiration (6). As 10–24% of healthy people may have the anatomical median arcuate ligament variant without true vascular obstruction (6), MALS can be misdiagnosed in patients with abdominal pain who undergo CT abdominal angiography to exclude vasculitis or thrombosis. Visceral Doppler is suggestive of MALS if the peak expiratory systolic velocity is > 200 cm/s, or the velocity is > 3:1 ratio compared to the adjacent aortic flow (7). The normal visceral arterial Doppler in this case was crucial in differentiating the respiration-related, physiological coeliac artery compression from coeliac artery thrombosis or MALS. The diagnosis of lupus enteritis in our case was corroborated by MRE. This was preferred over conventional CT enterography because of our patient’s young age and recent radiation exposure. MRE is currently used in assessing Crohn’s disease, particularly in younger patients, as a radiation-sparing alternative to CT enterography with
www.scandjrheumatol.dk
1. Zoller B, Li X, Sundquist J, Sundquist K. Risk of pulmonary embolism in patients with autoimmune disorders: a nationwide follow-up study from Sweden. Lancet 2012;379:244–9. 2. Zoller B, Li X, Sundquist J, Sundquist K. Risk of subsequent coronary heart disease in patients hospitalized for immunemediated diseases: a nationwide follow-up study from Sweden. PLoS One 2012;7:e33442. 3. Tektonidou MG, Laskari K, Panagiotakos DB, Moutsopoulos HM. Risk factors for thrombosis and primary thrombosis prevention in patients with systemic lupus erythematosus with or without antiphospholipid antibodies. Arthritis Rheum 2009;61:29–36. 4. Al-Homood IA. Thrombosis in systemic lupus erythematosus: a review article. ISRN Rheumatol 2012;2012:428269. 5. D’Cruz DP, Khamashta MA, Hughes GR. Systemic lupus erythematosus. Lancet 2007;369:587–96. 6. Horton KM, Talamini MA, Fishman EK. Median arcuate ligament syndrome: evaluation with CT angiography. Radiographics 2005;25:1177–82. 7. Ozel A, Toksoy G, Ozdogan O, Mahmutoglu AS, Karpat Z. Ultrasonographic diagnosis of median arcuate ligament syndrome: a report of two cases. Med Ultrason 2012;14:154–7. 8. Grand DJ, Beland M, Harris A. Magnetic resonance enterography. Radiol Clin North Am 2013;51:99–112. 9. Smith LW, Petri M. Lupus enteritis: an uncommon manifestation of systemic lupus erythematosus. J Clin Rheumatol 2013;19:84–6. 10. Tian XP, Zhang X. Gastrointestinal involvement in systemic lupus erythematosus: insight into pathogenesis, diagnosis and treatment. World J Gastroenterol 2010;16:2971–7.
Narainraj Kamalaraj, 60 Mulgray Avenue, Baulkham Hills, NSW 2153, Australia. E-mail: [email protected] Accepted 11 September 2014
