Scandinavian Journal of Rheumatology
ISSN: 0300-9742 (Print) 1502-7732 (Online) Journal homepage: http://www.tandfonline.com/loi/irhe20
Evidence for a clinical association between body mass index and malabsorption in patients with systemic sclerosis V Bishop, E Harrison, S Lal & AL Herrick To cite this article: V Bishop, E Harrison, S Lal & AL Herrick (2015) Evidence for a clinical association between body mass index and malabsorption in patients with systemic sclerosis, Scandinavian Journal of Rheumatology, 44:4, 341-342, DOI: 10.3109/03009742.2015.1026272 To link to this article: http://dx.doi.org/10.3109/03009742.2015.1026272
Published online: 30 Apr 2015.
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kidney function tests were all normal. A bone marrow biopsy revealed myeloid hyperplasia with a left shift and a scan with radiolabelled neutrophils did not identify a source of infection. After a few days, the patient experienced increasing pain and stiffness in ankles, knees, and hips. A transient pink rash lasting about 2 h on his chest (twice) and on his forearms (once) was witnessed by one of the treating physicians. A diagnosis of AOSD was made and the patient was started on oral methotrexate (15 mg per week) and prednisolone (60 mg per day) with prompt symptomatic relief. Oral anticoagulation was stopped after 6 months and a thrombophilia screen revealed a heterozygous prothrombin (PT) 20210 mutation with normal levels of natural anticoagulants, negative antiphospholipid antibodies, and normal factor V Leiden. The prednisolone was tapered after 2 months and later stopped. Methotrexate was stopped after 8 months of treatment. Twenty months after onset of his illness, he remains in remission. Coagulation activation in AOSD (1) may progress towards disseminated intravascular coagulation with multi-organ failure and digital autoamputation (3). Digital (4) and coronary artery occlusion (5), central retinal vein (6) and portal vein (7) thrombosis have been described in AOSD as well as a fatal pulmonary embolism (8). In these cases other ‘hits’ tilted the balance towards clot formation: the digital artery occlusion occurred during a vasculitic phase of AOSD (4), the coronary artery occlusion occurred post-partum (5), and the central retinal and portal vein thromboses were associated with antiphospholipid antibodies (6, 7). Likewise,
heterozygous PT 20210 represented a further tilt to the ‘haematology stress syndrome’ of chronic inflammatory diseases (9) and contributed to the unusual thromboembolic presentation of our patient. References 1. Gallistl S, Mangge H, Neuwirth G, Muntean W. Activation of the haemostatic system in children with juvenile rheumatoid arthritis correlates with disease activity. Thromb Res 1998;92:267–72. 2. Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H, Kashiwazaki S, et al. Preliminary criteria for classification of adult Still’s disease. J Rheumatol 1992;19:424–30. 3. Ames PRJ, Walker E, Aw D, Marshall D, de Villiers F, Staber M. Multi-organ failure in adult onset Still’s disease: a septic disguise. Clin Rheumatol 2009;28(Suppl 1):S3–6. 4. Carson RA. Digital artery thrombosis and vasculitis in juvenile rheumatoid arthritis. Can Med Assoc J 1973;109:384–6. 5. Parry G, Goudevenos J, Williams DO. Coronary thrombosis postpartum in a young woman with Still’s disease. Clin Cardiol 1992;15:305–7. 6. Andrews A, Hickling P. Thrombosis associated with antiphospholipid antibody in juvenile chronic arthritis. Lupus 1997;6:556–7. 7. Morita H, Nishiwaki H, Nagayama Y, Yoshimura A. Portal vein thrombosis in adult-onset Still’s disease: a case report and literature review. Rheumatol Int 2009;29:1515–18. 8. Al-Temimi FA, George P. Adult onset Still’s disease in Oman. Sultan Qaboos Univ Med J 2006;6:41–5. 9. Reizenstein P. The haematological stress syndrome. Br J Haematol 1979;43:329–33. Paul RJ Ames, Haemostasis & Thrombosis Department, St George’s Hospital, Blackshaw Road, London SW17 0QT, UK. E-mail: [email protected]
Accepted 17 February 2015
Evidence for a clinical association between body mass index and malabsorption in patients with systemic sclerosis V Bishop1, E Harrison1, S Lal2, AL Herrick1 1
Institute of Inflammation and Repair, Salford Royal NHS Foundation Trust, The University of Manchester, Manchester Academic Health Science Centre, and 2Intestinal Failure Unit, Salford Royal NHS Foundation Trust, Salford, UK
Studies involving patients with systemic sclerosis (SSc) demonstrate that a significant proportion are at risk of, or suffer from, malnutrition (1–3). Specifically, a study by the Canadian Scleroderma Research Group (CSRG) reported that almost 18% of 586 patients studied were at high risk of malnutrition, when assessed using the ‘Malnutrition Universal Screening Tool’ (‘MUST’) (1, 4). There is a need to better understand risk factors for, and associations of, malnutrition in patients with SSc, especially given that the gastrointestinal (GI) tract is commonly involved in patients with SSc (5). Any part of the GI tract may be involved (6). In the cohort
studied by the CSRG, 11% were suspected, by physicians, to have malabsorption (1). Using unadjusted odds ratios (ORs), the authors reported a significant association [OR 2.1, 95% confidence interval (CI) 1.3–3.5)] between ‘MUST’ scores and physician-assessed possible malabsorption. We conducted a pilot retrospective study to look for associations between malnutrition [using body mass index (BMI), one component of ‘MUST’ as a surrogate] and certain clinical features of SSc. Specifically, we set out to test the hypothesis that BMI would be reduced in patients with upper GI involvement and with malabsorption.
Table 1. Mean BMI values (kg/m2) for those patients with and without specific clinical and serological features.
Upper GI involvement Malabsorption Anti-centromere antibody positive Anti-topoisomerase antibody positive Limited cutaneous SSc subtype
Patients with specific features
Patients without specific features
24.92 5.24 (n ¼ 164) 22.06 5.05 (n ¼ 23) 24.82 5.78 (n ¼ 66) 24.75 5.28 (n ¼ 21) 25.03 5.18 (n ¼ 147)
24.69 4.44 (n ¼ 35) 25.24 5.00 (n ¼ 176) 24.99 4.75 (n ¼ 133) 24.75 5.08 (n ¼ 178) 24.43 4.89 (n ¼ 52)
0.8138 0.0047 0.8249 0.2943 0.4695
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Values given as mean standard deviation.
Malabsorption was diagnosed in patients with small intestinal bacterial overgrowth and/or dysmotility requiring home parenteral nutrition. The study was approved by the Salford and Trafford Local Research Ethics Committee. Data were collected from an annually updated clinical database: 199 patients had recent height and weight data available and were included in the analysis. An unpaired t-test was used to compare BMI in patients with and without upper GI involvement, malabsorption, and anticentromere and anti-topoisomerase antibody positivity. BMI was also compared between patients with limited cutaneous and diffuse cutaneous disease. The mean age of the 199 patients was 59 years (sd 11.5), and 163 (82%) were female. Of these 199, 147 (74%) patients had limited cutaneous and 52 (26%) diffuse cutaneous SSc, 164 (82%) had documented upper GI tract involvement, while 23 (12%) had malabsorption. The mean BMI of all 199 patients was 24.9 kg/m2 (sd 5.1). Patients with proven malabsorption had a lower mean BMI than those without (22.06 vs. 25.24 kg/m2, p ¼ 0.0047) (Table 1). However, there was no significant difference between the mean BMI of those patients with and without upper GI tract involvement. Nor did BMI differ between patients with and without positive anti-centromere or anti-topoisomerase antibodies, or between patients with limited and diffuse cutaneous SSc. Our findings therefore support those of the CSRG (1), suggesting an association between malnutrition and malabsorption. Although an association between upper GI involvement and low BMI or risk of malnutrition might be anticipated, this has not been clearly shown. Both the CSRG (1) and Caporali et al (2) found no significant association with dysphagia, vomiting, or reflux, but did note an association with early satiety. In addition, the CSRG reported an association with nausea (1). In our study, the lack of association between BMI and upper GI involvement may be due to the high prevalence of upper GI tract involvement and the inability (in the context of a retrospective study) to differentiate between types and severity of this GI involvement. Future studies should better define the extent, and stratify the severity of,
upper GI tract involvement to identify which key factors, in addition to malabsorption, contribute to malnutrition in patients with SSc. In our study, BMI did not differ with respect to autoantibody profile or disease subset, supporting the findings of Caporali et al (2). The absence of an association with anti-topoisomerase positivity was in accordance with the results of the study by Marie et al (7) of the associates of small intestinal bacterial overgrowth, a known cause of malabsorption. However, other studies have reported an association with disease subtype: the CSRG reported an association with the ‘MUST’ score (increased risk of malnutrition with diffuse cutaneous SSc) (1) while Kayser et al (8) also reported lower BMI in those with diffuse cutaneous SSc. Future, larger studies should take into account whether, in patients with SSc, other factors may influence BMI, including cardiorespiratory involvement, musculoskeletal disease (affecting food preparation ability), and corticosteroid therapy.
References 1. Baron M, Hudson M, Steele R; Canadian Scleroderma Research Group. Malnutrition is common in systemic sclerosis: results from the Canadian Scleroderma Research Group database. J Rheumatol 2009;36:2737–43. 2. Caporali R, Caccialanza R, Bonino C, Klersy C, Cereda E, Xoxi B, et al. Disease-related malnutrition in outpatients with systemic sclerosis. Clin Nutr 2012;31:666–71. 3. Krause L, Becker MO, Brueckner CS, Bellinghausen C-J, Becker C, Schneider U, et al. Nutritional status as marker for disease activity and severity predicting mortality in patients with systemic sclerosis. Ann Rheum Dis 2010;69:1951–7. 4. Stratton RJ, Hackston A, Longmore D, Dixon R, Price S, Stroud M, et al. Malnutrition in hospital outpatients and inpatients: prevalence, concurrent validity and ease of use of the ‘malnutrition universal screening tool’ (‘MUST’) for adults. Br J Nutr 2004;92:799–808. 5. Thoua NM, Bunce C, Brough G, Forbes A, Emmanuel AV, Denton CP. Assessment of gastrointestinal symptoms in patients with systemic sclerosis in a UK tertiary referral centre. Rheumatology (Oxford) 2010;49:1770–5. 6. Harrison E, Herrick AL, McLaughlin JT, Lal S. Malnutrition in systemic sclerosis. Rheumatology (Oxford) 2012;51:1747–56.
7. Marie I, Ducrotte P, Denis P, Menard J-F, Levesque H. Small intestinal bacterial overgrowth in systemic sclerosis. Rheumatology (Oxford) 2009;48:1314–19. 8. Kayser C, Martini L, Pinheiro M, Szejnfeld V, Marighela TF. Evaluation of nutritional status and dietary intake in women with systemic sclerosis. Rheumatology (Oxford) 2012;51(Suppl 2):ii85–6.
Ariane L Herrick, Clinical Sciences Building, Salford Royal NHS Foundation Trust, Salford M6 8HD, UK. E-mail: [email protected]
Accepted 3 March 2015
Hepatitis C-associated glomerulonephritis mimicking systemic lupus erythematosus
Downloaded by [Washington University in St Louis] at 19:39 08 November 2015
M Krajewska, D Rukasz, K Jakuszko, H Augustyniak-Bartosik, J Penar, Z Bednarz, M Klinger Department and Clinic of Nephrology and Transplantation Medicine, Wroclaw Medical University, Poland
Hepatitis C virus (HCV) infection is known to be responsible for many autoimmune reactions but its association with systemic lupus erythematosus (SLE) has not yet been established. We present the case of young female patient infected with HCV, suspected of SLE accompanied by severe nephrotic syndrome (NS) treated effectively with antiviral agents. The 25-year-old female patient was admitted to the Internal Medicine Department presenting with weakness, hypertension, oliguria, and massive peripheral oedema. Laboratory tests showed anaemia, leucopenia, and thrombocytopenia, impaired renal function, and the features of severe NS with active urinary sediment. Immunological tests revealed the presence of a high titre of antinuclear antibodies (ANA) and anti-doublestranded DNA (anti-dsDNA) antibodies (Abs). Screening viral tests revealed HCV infection with active replication and genotype 1 was established. Serum cryoglobulins were negative. Laboratory data are shown in Table 1. Renal biopsy diagnosed lupus nephritis class IV (membranoproliferative glomerulonephritis). Immunosuppressive
treatment was introduced: pulses of methylprednisolone intravenously at a total dose of 1.5 g followed by oral methyloprednisolone at a dose of 48 mg every second day. As a result her kidney function improved and proteinuria decreased. Moreover, peripheral blood count normalized, ANA titre diminished, and anti-dsDNA Abs became negative. A liver biopsy revealed chronic hepatitis lesions (hepatitis chronica minimalis) without features of autoimmune hepatitis (AIH). For the next year the dose of methyloprednisolone was gradually decreased to 12 mg every second day. Serum albumin and protein concentrations increased slightly despite preserved nephrotic range proteinuria. In the 17th month of observation the patient was referred to the Nephrology Department. Laboratory tests showed normal serum creatinine, persistent NS, and slightly increased ANA and anti-dsDNA Abs. Low immunological activity with high daily proteinuria accompanied by high HCV replication led us to believe that the renal disease was secondary to HCV infection. We decided to implement antiviral therapy containing pegylated interferon α-2a treatment (180 μg/week) and
Table 1. Laboratory results. Serum Serum Serum Daily RBC Haemoglobin WBC PLT ALT GGTP creatinine protein albumin proteinuria HCV RNA ANA Month (106/μL) (g/L) (103/μL) (103/μL) (U/L) (U/L) (mmol/L) (g/L) (g/L) (g) (copies/mL) (titre) 0 3.93 5 4.78 12 4.75 17 5.42 21 5.08 29 3.31 37 4.05 54 5.78 Normal 4.2–5.4
10.2 12.9 14.6 14.5 14.3 10.4 12.9 13.7 12–16
2.4 4.8 6.6 4.8 6.76 4.2 2.3 4.77 4–10
93 10 15 208 36 61 239 39 53 189 37 38 232 44 78 162 19 44 140 22 75 296 34 97 140–440 0–35 0–38
129.6 83.9 91.5 100.7 88.5 72.4 70.9 70.9 62–99
46 48 49 49 55 51 59 60 66–83
14 24 26 30 27 36 35 35–52
7.5 5.1 4.6 4.5 4.0 No data 2.0 Negative Negative
Positive 1.02 104 1.12 106 2.21 106 Negative Negative Negative Negative
1:1280 1:640 1:320 1:320 1:320 1:100 1:320 1:100 Negative
Anti-dsDNA Abs (IU/mL) 774.82 Negative Negative 104.67 162.83 52.14 80.16 63.77 < 100