Clinics and Practice 2011; volume 1:e89 Systemic lupus erythematosus- same patient. 1 Myositis (polymyositis PM or dermatomyositis DM) identifies a group of Correspondence: Faten Frikha, Department of myositis overlap syndrome: patients in whom the mascular weakness is Internal Medicine, Hospital of Hedi Chaker, 3029 report of 6 cases the principle clinical feature often associated Sfax, Tunisia. with muscle pain, tenderness and wasting, or E-mail: [email protected] Fatma Maazoun, 1 Faten Frikha, 1 other form of connective tissue diseases; the Key words: overlap syndrome, systemic lupus ery - Mouna Snoussi, 1 Neila Kaddour, 1 muscle biopsy generally demonstrates areas of thematosus, myositis, classification criteria, cor - Hatem Masmoudi, 2 Zouhir Bahloul 1 muscle fibre necrosis accompanied by intersti - ticosteroid. tial and/or perivascular cellular infiltrates. 1Department of Internal Medicine, Received for publication: 22 September 2011. Myositis associated with overlap syndromes is Hospital of Hedi Chaker; 2Laboratory of Accepted for publication: 12 October 2011. usually of paroxysmal variety and has been immunology, Hospital Habib Bourguiba, associated with one or another of connective This work is licensed under a Creative Commons Sfax, Tunisia tissue disorders [Systemic Sclerosis (SSc), Attribution NonCommercial 3.0 License (CC BY- Rheumatoid arthritis (RA), Sjögren’s syn - NC 3.0). drome or systemic lupus erythematous (SLE)]. ©Copyright F. Maazoun et al., 2011 Pearson and Bohan found an incidence of 21% Licensee PAGEPress, Italy Abstract 2 of this type of myositis. Myositis is a rare com - Clinics and Practice 2011; 1:e89 plication of systemic lupus erythematous 3,4 doi:10.4081/cp.2011.e89 The incidence of myositis in patients with occurring in almost 4-16% of cases of SLE 3,5,6 systemic lupus erythematosus (SLE) is low and such association is considered to be an among different series. Here we attempt to heterogeneous condition, sometimes less describe the main features of SLE/myositis severe, 7 sometimes similar 2,3 or even worse 8 Other muscle’s investigations including overlap syndrome. We retrospectively reviewed than the primary disease. electromyography (EMG) and muscle biopsy the medical records of 174 patients with SLE Herein we report a survey of 6 patients who findings were also noted. seen over 15-year period. All the patients ful - developed overlap syndrome of systemic lupus Patients with an overlap syndrome were filled the revised American Rheumatology erythematosus and myositis. We attempt to subclassified into those who had a monophasic Association criteria for SLE. Patients who met analysis the epidemiological, clinical, illness (a single episode of active disease), The Bohan and Peter criteria for definite immunological features and therapeutic man - relapsing-remitting disease (disease flares myositis were included in this study. Among agement of this rare condition, which further associated with disease-free periods), chronic those patients, six patients had an associated elucidation. progressive disease (evidence of active dis - myositis (3.4% overall). They were 6 women ease despite treatment) and remission. with a mean age of 29 years (20-41 years). At the initial evaluation, 3 patients (50%) were complained from myalgia, and all patients had Materials and Methods symmetrical muscle weakness (proximal mus - Results cle weakness in 6 cases with distal muscle We retrospectively reviewed the medical weakness in 2 cases). The muscle disease was records of patients with SLE attending the Among a large cohort of 174 SLE patients severe in 1 case. Involvements of muscles of Department of internal medicine of The (whom 162 were females with a frequency of the pharynx and upper esophagus were noted University Hédi Chaker Hospital (Sfax, 93%) who had attended the Department of in 4 patients (66.6%). The creatine kinase Tunisia) between January 1996 and December internal medicine, 6 patients were found to (CK) levels were elevated in 4 cases with a 2010. All these patients fulfilled The revised have overlap of SLE and idiopathic myositis mean rate of 2153.5 UI/L. The electromyogram American Rheumatology Association criteria giving a frequency of 3.4%. Although myalgia (EMG) revealed signs of myositis in 5 cases. (ACR) for SLE. 9 Patients who had an associat - was found as a feature of SLE in 15.8% , a true Muscle biopsy, performed in 5 patients, ed myositis were included in this study. myositis defined by the Bohan and Peter crite - revealed an inflammatory myopathy changes For myositis diagnosis, we used the criteria ria was found in only 3.4% of our SLE patients. in 4 cases. Antinuclear antibodies (ANA) were proposed by Bohan and Peter for definite The mean age at presentation was 29 years positive in all cases. All our patients were myositis [dermatomyositis (DM) or polymyosi - (range between 20 and 41 years) all the treated with high doses of corticosteroids with tis (PM)], 10 including symmetrical muscle patients were females. SLE and myositis were favorable outcome. Relapse of SLE disease had weakness, increase in serum muscle enzymes, diagnosed at the same time in 5 cases (83.3%). occurred in 2 patients. The association SLE- characteristic electromyographic pattern, Myositis had occurred 9 months after SLE diag - myositis is rare with heterogeneous presenta - signs of myositis proven by muscle biopsy and nosis in one case. tion. Through our observations and literature in case of DM typical cutaneous rash. data we will specify the characteristics of this For each case, we studied the demographic Clinical features association. data (gender, age at onset, duration of symp - Four patients (66.6%) were symptomatic for toms), clinical features, investigations, treat - more than 2 months prior to presentation ment details, and the response to treatment. (range between 2 and 8 months). Constitu- Laboratory findings including levels of tional symptoms were the most common fea - Introduction serum creatine kinase (CK), hematological tures at disease onset. They were present in 5 details and renal function were obtained. For patients. Five patients (83.3%) had fever and 3 The term overlap syndrome includes a large immunological data, antinuclear antibodies patients (50%). had general fatigue. At the group of conditions characterized by the coex - (ANA) using HEp2 cells, antibodies directed time of SLE diagnosis, 4 of our patients istence of signs, symptoms and immunological against native double stranded (ds) DNA, (66.6%) had mucocutaneous involvements. features of 2 or more connective tissue dis - extractable antigens [Ro(SSA), La(SSB), RNP, They were photosensitivity in 4 cases (66.6%), eases and occurring simultaneously in the Sm.] were recorded. mild mucosal ulceration in 3 cases (50%), [Clinics and Practice 2011; 1:e89] [page 189 ] Article malar rash in 2 cases (33.3%) and discoid SLE normal in one case. with SLE, true myositis is relatively rare as rash in 1 case (16.6%). Upon examination, 2 Renal biopsy was performed in 2 cases. It shown also by our study. 15 Myositis can occur patients (33.3%) had moderate arthritis (pain, disclosed aggressive diffuse proliferative lupus before, after SLE (case 2), or sporadically both swelling and tenderness in more than 2 joints nephritis (type IV) according to the World diseases can be present simultaneously (cases with some loss of function range of move - Health Organization WHO classification in one 1, 3, 4, 5 and 6 in our series). 14 ments), and 1 patient (16.6%) had pericardial case (case n°4), and showed type II lupus Several published studies had described the effusion. Neurological involvement was noted nephritis in the second case (case n°6). clinical characteristics of myositis in patients in 3 patients (50%): seizure in 2 patients and with SLE in some series 3,5,6,16 or in cases documented cerebral vasculitis in 1 patient. Treatment and clinical course reports. 4,14,17,18 Our lupus myositis patients Two patients were found to have urine abnor - All of our patients had been treated with oral were all female which is concordant to the pre - malities (33.3%). prednisone (1 mg/kg/day) for 6 weeks. Then, vious studies. 3,7,14,17,19 The study reported by At the time of initial evaluation, 3 patients the dose was gradually tapered to 10 mg/day. Foot et al. 6 suggested that lupus myositis was (50%) were complained from myalgia, and all Three patients had received intravenous pulse very similar to primary disease, in contrast to patients had symmetrical muscle weakness of methylprednisolone (1g per day for 3 days the milder descriptions of Fessel 16 and Tsokos (proximal muscle weakness in 6 cases with consecutively followed by high dose of oral et al. 5 were less than rigorous: all but one of distal muscle weakness in 2 cases). The mus - prednisone. The main indication being severe their patients with alleged myositis had a nor - cle disease was severe in 1 case with complete neurological involvement (cerebral vasculitis) mal serum CK, and only 5/18 subjects had a proximal and distal deficit in 1 case. in one case, severe myositis in one case and confirmatory muscle biopsy. Although it Involvements of muscles of the pharynx and proliferative lupus nephritis (type IV according remains unclear, some reports have indicated upper esophagus were noted in 4 patients to the WHO classification) in one case. The lat - that this overlap syndrome follows a benign (66.6%). It was a rapidly progressive dysphagia ter has also been treated with monthly intra - course and that the prognosis of myositis asso - in 4 cases and a dysphonia in 1 case. One venous Cyclophosphamide pulses in associa - ciated with SLE is reputed to be better than pri - patient had typical DM skin changes tion with corticosteroid therapy. mary myositis in terms of morbidity and (heliotrope rash) leading to the diagnosis of Over a mean follow up of 6 years (6 months response to therapy. 20-22 However, there have DM.