Scand J Rheumatol 2014;43:329–333
329
Whole-body magnetic resonance imaging in the assessment of muscular involvement in juvenile dermatomyositis/polymyositis patients TCM Castro1, H Lederman2, MT Terreri1, WI Caldana2, E Zanoteli3, MOE Hilário1
Scand J Rheumatol Downloaded from informahealthcare.com by Memorial University of Newfoundland on 01/28/15 For personal use only.
Department of 1Paediatrics, and 2Radiology, Federal University of São Paulo, and 3Department of Neurology, Medical School of the University of São Paulo, Brazil
Objectives: Our aim was to demonstrate the benefit of whole-body magnetic resonance imaging (WBMRI) as a diagnostic modality in the detection of muscle activity in juvenile dermatomyositis (JDM)/polymyositis (JPM) patients and to correlate these findings with clinical evaluation, laboratory examinations, nailfold capillaroscopy (NFC), and muscle biopsy. Method: Thirty-four patients aged 5.5 to 18.9 years with a diagnosis of JDM/JPM were prospectively evaluated using clinical examination, muscle enzyme determination, the Childhood Myositis Assessment Scale (CMAS), Disease Activity Score (DAS), Manual Muscle Testing (MMT), NFC, and WBMRI. An open muscle biopsy was performed if muscle disease activity was detected on WBMRI. Results: Disease activity was detected in WBMRI in four (11.7%) patients and confirmed by muscle biopsy. All four patients had elevation of at least one muscle enzyme and NFC showed scleroderma patterns in these patients. Conclusions: WBMRI allows us to evaluate the extent and symmetry of muscle disease and inflammatory activity. NFC is an important additional examination to assess disease activity.
Juvenile dermatomyositis (JDM) is a rare autoimmune disease characterized by a skin rash with purple discoloration over the eyelids (heliotrope rash) and erythematous patchy skin on extensor surfaces (Gottron’s papules), proximal muscle weakness, raised muscle enzymes, myopathic changes on electromyography (EMG), and typical muscle biopsy (1–3). According to the Bohan and Peter criteria, a definite diagnosis is made if a characteristic skin rash is present in combination with three of the four criteria (4). Changes in clinical practice over time have resulted in many clinicians using non-invasive techniques, such as magnetic resonance imaging (MRI), in place of EMG and muscle biopsy (3). The advent of short tau inversion recovery (STIR) whole-body sequences allows rapid MRI of the entire body in a reasonable time, giving excellent detail of muscle inflammation (5). Our aim was to demonstrate the benefit of STIR wholebody MRI (WBMRI) as a diagnostic modality in the detection of muscle disease activity in JDM and to correlate these findings with clinical evaluation including muscle function and strength tests, laboratory examinations, nailfold capillaroscopy (NFC), and muscle biopsy.
Tania Caroline Monteiro de Castro, Rua Camilo Nader, 315, apartamento 22, São Paulo, CEP 05688-032, Brazil. E-mail: [email protected] Accepted 19 November 2013
Method Patients Thirty-four patients (24 girls and 10 boys) aged 5.5 to 18.9 years (median age 12.2 years) with a diagnosis of JDM (n = 32) or juvenile polymyositis (JPM; n = 2) according to Bohan and Peter criteria were prospectively evaluated at any point during their illness course. All patients with active or inactive disease were followed up at the Paediatric Rheumatology Unit of the Federal University of São Paulo o. All patients were evaluated for demographic data and the use of corticosteroids and other immunosuppressive drugs. The ethical committee approved this study (no. 0776/10) and written informed consent was obtained from each participant aged > 12 years and from at least one parent or legal guardian of all participants.
Muscle function and strength tests, and disease activity Muscle function was assessed by the Childhood Myositis Assessment Scale (CMAS) (6) with scores ranging from 0 to 52. Muscle strength was assessed by Manual Muscle Testing (MMT) using a 10-point scale ranging from 0 to 80 (7). JDM activity was evaluated by the Disease Activity Score (DAS), which ranged from 0 to 20 (8).
© 2014 Informa Healthcare on license from Scandinavian Rheumatology Research Foundation DOI: 10.3109/03009742.2013.868509
www.scandjrheumatol.dk
330
TCM Castro et al
B
A
Scand J Rheumatol Downloaded from informahealthcare.com by Memorial University of Newfoundland on 01/28/15 For personal use only.
Figure 1. In a 9-year-old boy with biopsy-proven JDM, coronal STIR WBMRI shows inflammation in (A) supraspinal, infraspinal (long arrows), and deltoid (short arrow) muscles and (B) triceps sural muscle.
NFC NFC was performed by the same researcher (MTT) who was blinded to the level of disease activity. The parameters were analysed according to the methodology and definitions described elsewhere (9). Examination findings were considered abnormal if ectasia or giant capillaries were observed, if there was any dropout, and/or if there were diffuse haemorrhages in two or more fingers. A scleroderma (SD) pattern was defined as the presence of capillary ectasia or giant capillaries and avascular lesions indicating capillary dropout. WBMRI All WBMR images were acquired on a 1.5-T unit (Intera Achieva, Philips, Best, The Netherlands) in the coronal plane using a turbo STIR technique with a repetition time (TR) of 4900 ms, an echo time (TE) of 66 ms, an inversion time (TI) of 160 ms, a field of view (FOV) of 48 cm, and a slice thickness of 7 mm. An experienced musculoskeletal radiologist analysed the images and muscle disease activity was represented by high signal intensity on STIR images. Muscle biopsy Muscle fragments collected from open muscle biopsy in the biceps brachialis were frozen in isopentane pre-cooled in liquid nitrogen. Sequential cryostat sections were
A
B
Figure 3. Coronal STIR WBMRI in a 15-year-old girl (the same patient as in Figure 2) showing diffuse distribution of muscle inflammation.
stained for routine and histochemical reactions, and immunostained for dysferlin, CD4+ and CD8+ T lymphocytes, CD68+ macrophage, and class I major histocompatibility complex (MHC) (10). We considered that the disease was active when muscle necrosis, fibre regeneration, endomysial mononuclear cell infiltrates, infiltrates with positive cells for macrophages and CD8+ lymphocytes, and expression of class I MHC antigens on the muscle fibres were detected.
C
Figure 2. Coronal STIR WBMRI in a 15-year-old girl showing oedema in (A) deltoid (wide arrow) and biceps (narrow arrow) muscles, (B) adductor muscles, and (C) quadriceps muscle.
www.scandjrheumatol.dk
F F M M F F F M M F F F F M F F F F F M F F M F M F F F M M F F F F
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34
16.5 15.3 13.2 14.3 15.1 17.3 12.3 17.7 15.1 10.7 16.5 12.5 10.7 6.8 9.4 10.8 14.6 12.0 8.0 14.5 12.1 10.0 9.2 18.9 6.0 15.3 6.3 5.5 6.9 10.0 8.2 11.0 12.1 6.6
Age (years)
10.6 5.2 2.3 9.2 1.7 7.6 6.7 3.8 6.8 5.8 10.1 8.0 7.2 3.5 6.2 6.1 10.4 3.0 4.8 11.9 10.3 8.8 7.0 5.0 4.4 15.1 2.0 1.0 3.4 7.8 6.2 10.8 11.6 5.8
Age at onset (years) 5.9 10.0 10.9 5.1 13.3 9.7 5.6 13.9 8.3 4.9 6.4 4.5 3.6 3.3 3.2 4.7 4.2 9.0 3.2 2.6 1.7 1.2 2.1 13.9 1.67 0.19 4.27 4.48 3.51 2.17 2.00 0.20 0.49 0.85
Disease duration (years) 3.0 24.0 0.3 3.0 10.0 16.0 8.0 4.0 7.0 6.0 27.0 20.0 7.0 9.0 9.0 9.0 70.0 90.0 37.0 6.0 10.0 15.0 6.0 5.0 2.0 ND 20.0 48.0 4.0 2.0 32.0 8.0 20.0 2.0
ESR (mm/h) 2.36 0.12 1.7 0.58 0.31 0.26 1.84 ND 4.3 ND 8.84 1.12 1.63 2.6 0.000 1.5 5.000 ND 1.81 2.11 2.24 0.12 0.2 2.000 4.4 5.38 4.7 4.000 1.75 0.23 0.8 0.3 0.7
329
Whole-body magnetic resonance imaging in the assessment of muscular involvement in juvenile dermatomyositis/polymyositis patients TCM Castro1, H Lederman2, MT Terreri1, WI Caldana2, E Zanoteli3, MOE Hilário1
Scand J Rheumatol Downloaded from informahealthcare.com by Memorial University of Newfoundland on 01/28/15 For personal use only.
Department of 1Paediatrics, and 2Radiology, Federal University of São Paulo, and 3Department of Neurology, Medical School of the University of São Paulo, Brazil
Objectives: Our aim was to demonstrate the benefit of whole-body magnetic resonance imaging (WBMRI) as a diagnostic modality in the detection of muscle activity in juvenile dermatomyositis (JDM)/polymyositis (JPM) patients and to correlate these findings with clinical evaluation, laboratory examinations, nailfold capillaroscopy (NFC), and muscle biopsy. Method: Thirty-four patients aged 5.5 to 18.9 years with a diagnosis of JDM/JPM were prospectively evaluated using clinical examination, muscle enzyme determination, the Childhood Myositis Assessment Scale (CMAS), Disease Activity Score (DAS), Manual Muscle Testing (MMT), NFC, and WBMRI. An open muscle biopsy was performed if muscle disease activity was detected on WBMRI. Results: Disease activity was detected in WBMRI in four (11.7%) patients and confirmed by muscle biopsy. All four patients had elevation of at least one muscle enzyme and NFC showed scleroderma patterns in these patients. Conclusions: WBMRI allows us to evaluate the extent and symmetry of muscle disease and inflammatory activity. NFC is an important additional examination to assess disease activity.
Juvenile dermatomyositis (JDM) is a rare autoimmune disease characterized by a skin rash with purple discoloration over the eyelids (heliotrope rash) and erythematous patchy skin on extensor surfaces (Gottron’s papules), proximal muscle weakness, raised muscle enzymes, myopathic changes on electromyography (EMG), and typical muscle biopsy (1–3). According to the Bohan and Peter criteria, a definite diagnosis is made if a characteristic skin rash is present in combination with three of the four criteria (4). Changes in clinical practice over time have resulted in many clinicians using non-invasive techniques, such as magnetic resonance imaging (MRI), in place of EMG and muscle biopsy (3). The advent of short tau inversion recovery (STIR) whole-body sequences allows rapid MRI of the entire body in a reasonable time, giving excellent detail of muscle inflammation (5). Our aim was to demonstrate the benefit of STIR wholebody MRI (WBMRI) as a diagnostic modality in the detection of muscle disease activity in JDM and to correlate these findings with clinical evaluation including muscle function and strength tests, laboratory examinations, nailfold capillaroscopy (NFC), and muscle biopsy.
Tania Caroline Monteiro de Castro, Rua Camilo Nader, 315, apartamento 22, São Paulo, CEP 05688-032, Brazil. E-mail: [email protected] Accepted 19 November 2013
Method Patients Thirty-four patients (24 girls and 10 boys) aged 5.5 to 18.9 years (median age 12.2 years) with a diagnosis of JDM (n = 32) or juvenile polymyositis (JPM; n = 2) according to Bohan and Peter criteria were prospectively evaluated at any point during their illness course. All patients with active or inactive disease were followed up at the Paediatric Rheumatology Unit of the Federal University of São Paulo o. All patients were evaluated for demographic data and the use of corticosteroids and other immunosuppressive drugs. The ethical committee approved this study (no. 0776/10) and written informed consent was obtained from each participant aged > 12 years and from at least one parent or legal guardian of all participants.
Muscle function and strength tests, and disease activity Muscle function was assessed by the Childhood Myositis Assessment Scale (CMAS) (6) with scores ranging from 0 to 52. Muscle strength was assessed by Manual Muscle Testing (MMT) using a 10-point scale ranging from 0 to 80 (7). JDM activity was evaluated by the Disease Activity Score (DAS), which ranged from 0 to 20 (8).
© 2014 Informa Healthcare on license from Scandinavian Rheumatology Research Foundation DOI: 10.3109/03009742.2013.868509
www.scandjrheumatol.dk
330
TCM Castro et al
B
A
Scand J Rheumatol Downloaded from informahealthcare.com by Memorial University of Newfoundland on 01/28/15 For personal use only.
Figure 1. In a 9-year-old boy with biopsy-proven JDM, coronal STIR WBMRI shows inflammation in (A) supraspinal, infraspinal (long arrows), and deltoid (short arrow) muscles and (B) triceps sural muscle.
NFC NFC was performed by the same researcher (MTT) who was blinded to the level of disease activity. The parameters were analysed according to the methodology and definitions described elsewhere (9). Examination findings were considered abnormal if ectasia or giant capillaries were observed, if there was any dropout, and/or if there were diffuse haemorrhages in two or more fingers. A scleroderma (SD) pattern was defined as the presence of capillary ectasia or giant capillaries and avascular lesions indicating capillary dropout. WBMRI All WBMR images were acquired on a 1.5-T unit (Intera Achieva, Philips, Best, The Netherlands) in the coronal plane using a turbo STIR technique with a repetition time (TR) of 4900 ms, an echo time (TE) of 66 ms, an inversion time (TI) of 160 ms, a field of view (FOV) of 48 cm, and a slice thickness of 7 mm. An experienced musculoskeletal radiologist analysed the images and muscle disease activity was represented by high signal intensity on STIR images. Muscle biopsy Muscle fragments collected from open muscle biopsy in the biceps brachialis were frozen in isopentane pre-cooled in liquid nitrogen. Sequential cryostat sections were
A
B
Figure 3. Coronal STIR WBMRI in a 15-year-old girl (the same patient as in Figure 2) showing diffuse distribution of muscle inflammation.
stained for routine and histochemical reactions, and immunostained for dysferlin, CD4+ and CD8+ T lymphocytes, CD68+ macrophage, and class I major histocompatibility complex (MHC) (10). We considered that the disease was active when muscle necrosis, fibre regeneration, endomysial mononuclear cell infiltrates, infiltrates with positive cells for macrophages and CD8+ lymphocytes, and expression of class I MHC antigens on the muscle fibres were detected.
C
Figure 2. Coronal STIR WBMRI in a 15-year-old girl showing oedema in (A) deltoid (wide arrow) and biceps (narrow arrow) muscles, (B) adductor muscles, and (C) quadriceps muscle.
www.scandjrheumatol.dk
F F M M F F F M M F F F F M F F F F F M F F M F M F F F M M F F F F
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34
16.5 15.3 13.2 14.3 15.1 17.3 12.3 17.7 15.1 10.7 16.5 12.5 10.7 6.8 9.4 10.8 14.6 12.0 8.0 14.5 12.1 10.0 9.2 18.9 6.0 15.3 6.3 5.5 6.9 10.0 8.2 11.0 12.1 6.6
Age (years)
10.6 5.2 2.3 9.2 1.7 7.6 6.7 3.8 6.8 5.8 10.1 8.0 7.2 3.5 6.2 6.1 10.4 3.0 4.8 11.9 10.3 8.8 7.0 5.0 4.4 15.1 2.0 1.0 3.4 7.8 6.2 10.8 11.6 5.8
Age at onset (years) 5.9 10.0 10.9 5.1 13.3 9.7 5.6 13.9 8.3 4.9 6.4 4.5 3.6 3.3 3.2 4.7 4.2 9.0 3.2 2.6 1.7 1.2 2.1 13.9 1.67 0.19 4.27 4.48 3.51 2.17 2.00 0.20 0.49 0.85
Disease duration (years) 3.0 24.0 0.3 3.0 10.0 16.0 8.0 4.0 7.0 6.0 27.0 20.0 7.0 9.0 9.0 9.0 70.0 90.0 37.0 6.0 10.0 15.0 6.0 5.0 2.0 ND 20.0 48.0 4.0 2.0 32.0 8.0 20.0 2.0
ESR (mm/h) 2.36 0.12 1.7 0.58 0.31 0.26 1.84 ND 4.3 ND 8.84 1.12 1.63 2.6 0.000 1.5 5.000 ND 1.81 2.11 2.24 0.12 0.2 2.000 4.4 5.38 4.7 4.000 1.75 0.23 0.8 0.3 0.7
