Anti-p155/140 Antibody-positive Dermatomyositis with Metastasis Origina­ting from an Unknown Site

Abstract

Dermatomyositis (DM) is a systemic inflammatory myopathy with characteristic cutaneous manifestations (a heliotrope rash, Gottron's papules, paronychial erythema and nailfold bleeding) and is often associated with interstitial lung disease and internal malignancy. Thus far, some autoantibodies specific for myositis have been discovered, including antibodies to aminoacyl-tRNA synthetases (ARS), anti-Mi-2 antibodies, anti-CADM 140 antibody, anti-pl55/140 antibody and others (1-3). The various autoantibody-positive subgroups of DM vary in their clinical features. Of these myositis-specific autoantibodies, the anti-p 155/140 antibody is a 155-kDa reactive nuclear protein relevant to cancer-associated DM (1, 4-8). However, the frequency of malignancies in patients with anti-p 155/140 antibody is undefined because no large epidemiological studies have been undertaken. We describe here a patient with anti-pl 55/140 antibodypositive DM who had a poorly differentiated metastatic adenocarcinoma; however, the primary tumour could not be identified despite comprehensive examination. CASE REPORTA 57-year-old man presented with refractory erythema on the hands and face, muscle weakness and dysphagia. Two months before consultation, he had had erythema on the face, which had spread to the precordium and limbs.At the first presentation the patient had a typical heliotrope rash, Gottron's papules, paronychial erythema, nailfold bleeding and hyperkeratotic erythema over the elbow (Fig. 1). Blood examination revealed a high erythrocyte sedimentation rate (62 mm/h), high levels of lactate dehydrogenase (LDH) (295 IU/1), C-reactive protein (CRP) (8.13 mg/dl), creatine kinase (CK) (863 IU/1; reference values: 50-200 IU/1), myoglobin (240 ng/ ml) and aldolase (8.6 U/1). The antinuclear antibody titre was positive at 1:40 with a homogeneous and speckled pattern. As for tumour markers, carcinoembryonic antigen (CEA) was high at 115 ng/ml (reference values: < 5.0 ng/ml). He was later found to be positive for anti-pl 55/140 antibody by an immunoprecipitation study performed using extracts of the leukaemia cell line, K562 (4, 9) (Fig. 2). Chest computerized tomography (CT) revealed aspiration pneumonia. However, there were no signs of interstitial pneumonia.Biopsy specimens were obtained from the left deltoid muscle and the Gottron's papule on the fifth metacarpophalangeal joint of the left hand. Histology of the muscle showed inflatnmatory infiltration of mainly lymphocytes around the muscle fibres. The muscle fibres showed necrotic changes, including size irregularities and reduced staining. The skin biopsy showed hyperkeratosis, thickening of the granular layer, slight lymphocyte infiltration and pigment incontinence at the dermo-epidermal junction.On the basis of the clinical and pathological findings, we diagnosed this case as DM. Taking into account the high CEA, upper gastrointestinal endoscopy and colonoscopy, hepatic and mamActa Derm Venereol 9i Fig. 1. (a) Heliotrope rash and (b) Gottron's papules, paronychial erythema, and nailfo...

Four dermatomyositis-specific autoantibodies—anti-TIF1γ, anti-NXP2, anti-SAE and anti-MDA5—in adult and juvenile patients with idiopathic inflammatory myopathies in a Hungarian cohort

“…Ohashi investigated five cases of DM with anti-TIF1γ positivity, 80% of which had a malignant tumour of some kind. In contrast to our results, they observed two large-cell lung carcinoma, one poorly differentiated adenocarcinoma and one metastatic hepatocarcinoma [49]. In 2011, Zahr and Baer also stated that the anti-TIF1γ antibody has a strong predictive value for malignancy in adult patients [51].…”

“…Three adult DM patients were smokers at the time of disease onset. A comparison of our results of the frequency of this antibody with the results of other studies [46][47][48][49][50] can be seen in Table 1. The clinically relevant data of our patients with anti-TIF1γ positivity can be seen in Table 2.…”

“…Targoff [46] Kaji [47] Gunawardena [48] Ohashi [49] Hoshino [50] Our TIF1γ is DM together with malignancy in adult patients. In juvenile DM patients, antibodies for TIF-1γ are not associated with malignancy, but rather, with skin ulcerations and more extensive cutaneous diseases [33].…”

Idiopathic Inflammatory Myopathies and Malignancy: a Comprehensive Review

“…The 140-kDa proteins were identified as other members of the TIF-1 family [69] (TIF-1α, TIF-1β). The frequency of anti-TIF-1γ ranges from 8 to 21 % in adults with dermatomyositis and 15 to 30 % of juvenile dermatomyositis patients [59,62,[65][66][67][70][71][72]. Of the adult patients with this autoantibody, 38-80 % developed malignancy at some point prior to or after the diagnosis of myositis.…”

The specific features of the onset, clinical picture, steroid responsiveness of paraneoplastic myositis

“…До недавнего времени существовало мнение об отсутствии таковых при ПНП-миозите. Однако последние исследова-ния позволяют обсуждать роль антител к протеину 155/140 как специфических маркеров ПНП-миозита, что имеет как диагностическое (облегчающее скрининг при онкопо-иске), так и прогностическое значение [9][10][11][12][13]. Так, N. Ikeda и соавт.…”