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Inflammatory myopathy and autoimmune hepatitis in a patient with a flare of systemic lupus erythematosus: An exceptional association.


In systemic lupus erythematosus (SLE), the musculoskeletal manifestations are the most frequent manifestations, arthritis and arthralgias being the most common. Myalgias and muscular weakness are not uncommon, while inflammatory myopathies and polymyositis in particular are less frequent. Apart from the overlap with authentic inflammatory myopathies, cases of steroid myopathy, hydroxychloroquine-induced myopathy, and even vacuolar myopathy, have been reported (1). Digestive manifestations are less frequent in SLE, occurring in only 2%-30% of patients. Among these cases, the association of SLE with autoimmune hepatitis (AIH) is rare, with only a few cases being documented in the medical literature (2). In this manuscript, we present the case of a patient diagnosed with SLE suffering from a flare of the disease with simultaneous hepatic and muscular inflammatory involvement.

Case Presentation

A 46-year-old woman from Uruguay was diagnosed with SLE 22 years ago. She initially presented with bilateral hand polyarthritis and, at the time of diagnosis, she fulfilled clinical and analytical criteria for SLE (arthritis, presence of ANA, anti-dsDNA, and anti-Sm antibodies, photosensitivity, malar rash, and oral ulcers). In the years before moving to Barcelona, she had only received treatment with corticosteroids occasionally. In 2003, she received follow-up care in the Rheumatology Department at the Hospital del Mar/Pare de Salut Mar (Barcelona), where she started treatment with Dolquine (hydroxychloroquine) at a dose of 400 mg/day Corticotherapy was withdrawn, with no need for reintroduction at any time during her follow-up. With this therapy, the patient remained clinically and analytically stable, with no new flares or presence of another symptomatology attributable to SLE. We would like to highlight the persistent negativization of anti-dsDNA and of the rest of autoantibodies since 2007.

In December 2014, the patient was admitted to the emergency department due to fever that had lasted for three days, associated with arthralgias in proximal interphalangeal joints and both wrists, discrete malar rash, and pain focused in the region of the left quadriceps, with slight muscle weakness as well as pain at the palpation of the mentioned zone. Given these findings, the patient was admitted at the Rheumatology Department with the suspicion of an SLE flare. Serological findings revealed ANA 1/320, negative anti-dsDNA, and positivity for anti-Sm and anti-RNP antibodies, no proteinuria or hematuria, and mild lymphopenia (1050 cells/L) as the only alteration of the hemogram. Muscle enzymes were elevated (creatinine kinases [CK] of 309 IU/L and aldolase of 12.8 IU/L), and there was an increase in acute phase reactants, with an ESR of 34 mm/h, PCR of 4.09 mg/dL, and ferritin of 614 ng/mL There was also an increase in hepatic enzymes (GOT 694 IU/L, GPT 1044 IU/L, FA 130 IU/L). The serologies of the hepatotropic viruses (HAV, HBV, HCV) as well as the most common antibodies associated with autoimmune hepatitis (ASMA, anti-LKM1, anti-SLA, and anti-LP) were negative. In terms of complementary examinations, an MRI (magnetic resonance imaging) of the lower limbs was performed, which showed extensive muscular involvement of the left guadriceps, affecting the intermedius vastus and partially the lateralis and medialis, compatible with edema, inflammatory changes, or myositis (Figure 1, 2).

Three tissue fragments were obtained by muscle biopsy. The results of this study were consistent with an inflammatory myositis (Figure 3, 4). In view of the possibility of concomitant hepatic involvement, a percutaneous liver biopsy was performed, and two tissue cylinders were obtained and submitted for histological study. The biopsy results showed a pattern of lobule and portal hepatitis with plasma cell aggregates compatible, in the clinical context, with an active autoimmune hepatitis and without signs of chronicity (Figure 5, 6). Prednisone was started at a dose of 1 mg/kg (60 mg/day). Following the American guidelines for the clinical management of autoimmune hepatitis in young adult patients, no immunosuppressant treatment was applied at the beginning. Nevertheless, after a new increase in the values of muscular and liver enzymes during the descending regimen of corticosteroids, treatment with azathioprine at a dose of 50 mg/24 h was started, pending the results of the thiopurine methyltransferase (TPMT) polymorphism studies.


To date, the current case represents the first reported case of an overlap involving systemic lupus erythematosus, autoimmune hepatitis, and inflammatory myopathy Autoimmune liver disease may be associated with extrahepatic inflammatory manifestations (3). Several associations between autoimmune hepatitis, and polymyositis/dermatomyositis with other diseases have been documented: with thyroiditis and antiphospholipid syndrome; systemic sclerosis and cerebral vasculitis; primary biliary cirrhosis and thrombocytopenic purpura; myasthenia gravis and thymoma; membranous nephropathy and Sjogren's syndrome; and, finally, with systemic sclerosis and sarcoidosis of mediastinal lymphoid nodules (3-8). In the case described, the GOT/GPT ratio was not indicative of an acute process exclusively. Muscle pain in the lower extremities lead to an MRI of the lower limbs, with findings indicating focal myositis of the guadriceps muscle, results later confirmed by the muscle biopsy.

The diagnosis of autoimmune hepatitis was made by combining clinical, analytical (absence of other etiologies such as hepatotrope virus positivity, enolic intake or recently new introduced drugs) and histological data (liver biopsy compatible with presence of plasma cell aggregates in the portal regions). The pretreatment index for detecting autoimmune hepatitis scored 13 points (9).

Currently, the patient's progress is satisfactory, with azathioprine applied at a dose of 50 mg/24 h and hydroxychloroguine 400 mg/24 h. At present, she is stable without elevated liver enzymes or myalgias suggestive of persistent inflammatory myopathy.


When assessing a patient with SLE and evidence of clinical activity (acute flare) with elevated transaminases, it is essential to examine muscle enzymes and to rule out a possible myopathic involvement, isolated or even concomitant with autoimmune hepatitis, as in the case presented.

Informed Consent: Written informed consent was obtained from the patient who participated in this study.

Peer-review: Externally peer-reviewed.

Author Contributions: Concept - T.C.S.M., I.C.B., C.P.G., J.G.B., J.M.; Design - T.C.S.M., I.C.B., C.P.G., J.G.B., J.M.; Supervision T.C.S.M., I.C.B., C.P.G., J.G.B., J.M.; Resources - T.C.S.M., I.C.B., C.P.G., J.G.B., J.M.; Materials -T.C.S.M., I.C.B., C.P.G., J.G.B., J.M.; Data Collection and/or Processing - T.C.S.M., I.C.B., C.P.G., J.G.B., J.M.; Analysis and/or Interpretation - T.C.S.M., I.C.B., C.P.G., J.G.B., J.M.; Literature Search - T.C.S.M., I.C.B., C.P.G., J.G.B., J.M.; Writing Manuscript - T.C.S.M., I.C.B., C.P.G., J.G.B., J.M.; Critical Review-T.C.S.M., I.C.B., C.P.G., J.G.B., J.M.

Conflict of Interest: The authors have no conflict of interest to declare.

Financial Disclosure: The authors declared that this study has received no financial support.


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(2.) Beisel C, Weiler-Normann C, Teufel A, Lohse AW. Association of autoimmune hepatitis and systemic lupus erythematosus: a case series and review of the literature. World J Gastroenterol 2014;20: 12662-7. [CrossRef]

(3.) Pamfil C, Candrea E, Berki E, Popov HI, Radu PI, Rednic S. Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome associated with dermatomyositis, autoimmune thyroiditis and antiphospholipid syndrome. J Gastrointestin Liver Dis 2015; 24:101-4.

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(5.) Kurihara Y, Shishido T, Oku K, Takamatsu M, Ishiguro H, Suzuki A, et al.Polymyositis associated with autoimmunehepatitis, primary biliary cirrhosis, and autoimmune thrombocytopenic purpura. Mod Rheumatol 2011; 21: 325-9. [CrossRef]

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(7.) Stefanidis I, Giannopoulou M, Liakopoulos V, Dovas S, Karasavvidou F, Zachou K. A case of membranous nephropathy associated with Sjogren syndrome, polymyositis and autoimmune hepatitis. Clin Nephrol 2008; 70: 245-50. [CrossRef]

(8.) Lis-Swiety A, Brzezinska-Wcislo L, Pierzchala E, Wcislo-Dziadecka D. Systemic sclerosis-polymyositis overlap syndrome accompanied by autoimmune hepatitis and sarcoidosis of mediastinal lymphnodes. J Eur Acad Dermatol Venereol 2006;20:107-108. [CrossRef]

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Tarek Carlos Salman-Monte (1), Irene Carrion-Barbera (1), Carolina Perez Garcia (1), Javier Gimeno Beltran (2), Jordi Monfort (1)

(1) Department of Rheumatology, Hospital del Mar-Pare de Salut Mar, Barcelona, Spain

(2) Department of Pathology, Hospital del Mar - Parc de Salut Mar, Barcelona, Spain

Address for Correspondence: TarekCarlos Salman-Monte, Department of Rheumatology, Hospital del Mar- Parc de Salut Mar, Barcelona, Spain


Submitted: 14August 2017

Accepted: 21 September 2017

Available Online Date: 22 January 2018

DOI: 10.5152/eurjrheum.2018.17135
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Article Details
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Title Annotation:Case Report
Author:Salman-Monte, Tarek Carlos; Carrion-Barbera, Irene; Garcia, Carolina Perez; Beltran, Javier Gimeno;
Publication:European Journal of Rheumatology
Date:Sep 1, 2018
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