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Antisynthetase Syndrome Positive for Anti-Threonyl- Trna Synthetase (Anti-PL7) Antibodies

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Antisynthetase Syndrome Positive for Anti-Threonyl- Trna Synthetase (Anti-PL7) Antibodies 6 Gieffers J, Fullgraf H, Jahn J, et al. Chlamydia pneumoniae infection 10 Hammerschlag MR, Chirgwin K, Roblin PM, et al. Persistent in circulating human monocytes is refractory to antibiotic infection with Chlamydia pneumoniae following acute respiratory treatment. Circulation 2001; 103: 351–356. illness. Clin Infect Dis 1992; 14: 178–182. 7 Boman J, Gaydos CA. Polymerase chain reaction detection of 11 Miyashita N, Niki Y, Nakajima M, et al. Prevalence of asympto- Chlamydia pneumoniae in circulating white blood cells. J Infect Dis matic infection with Chlamydia pneumoniae in subjectively healthy 2000; 181: Suppl. 3, S452–S454. adults. Chest 2001; 119: 1416–1419. 8 Yamaguchi H, Yamada M, Uruma T, et al. Prevalence of viable 12 Blasi F, Damato S, Cosentini R, et al. Chlamydia pneumoniae and Chlamydia pneumoniae in peripheral blood mononuclear cells of chronic bronchitis: association with severity and bacterial clear- healthy blood donors. Transfusion 2004; 44: 1072–1078. ance following treatment. Thorax 2002; 57: 672–676. 9 Boman J, Soderberg S, Forsberg J, et al. High prevalence of 13 Wolf K, Fischer E, Hackstadt T. Degradation of Chlamydia pneumoniae Chlamydia pneumoniae DNA in peripheral blood mononuclear cells by peripheral blood monocytic cells. Infect Immun 2005; 73: 4560–4570. in patients with cardiovascular disease and in middle-aged blood donors. J Infect Dis 1998; 178: 274–277. DOI: 10.1183/09031936.00102310 Antisynthetase syndrome positive for anti-threonyl- tRNA synthetase (anti-PL7) antibodies To the Editors: Pulmonary hypertension was suspected by echocardiography when systolic pulmonary arterial pressure was .40 mmHg. Antisynthetase syndrome (ASS) is characterised by an inflam- matory myositis associated with interstitial lung disease (ILD) For the follow-up, we defined pulmonary aggravation/improve- and antisynthetase antibodies. Other symptoms, including ment as an increase/decrease in dyspnoea, according to New arthritis, Raynaud’s phenomenon and mechanic’s hands, are York Heart Association (NYHA) stages and/or deteriorating/ also associated with ASS. Several antisynthetase antibodies [1] improving pulmonary function tests (.10% decrease/increase have been described, with anti-Jo1 being the most common. in carbon monoxide transfer factor and/or total lung capacity Little is known about the clinical manifestations of ASS asso- between the first and the last tests). ciated with anti-threonyl-tRNA synthetase antibodies (anti- This study was approved by hospital local ethical committees. PL7) [2–4], most probably because anti-PL7 antibodies are The patients of this study are anonymously reported and in particularly rare (5% of myositis [5]) and have not been accordance with the French law, a patient consent was required. routinely researched in the past. The mean age at the first sign of clinical symptoms was 56.3 yrs We conducted this retrospective multicentric study to describe (range 31–71 yrs). Three patients had interrupted smoking the clinical, radiographic and biological manifestations of ASS habits; none had known toxic environmental exposure or any in 12 patients. family history of ILD. This retrospective study was conducted in five university All patients presented with ILD at the onset of the disease. hospitals. The patients were followed from 2000–2010 in the Muscular symptoms occurred in half of the patients. Fever and Depts of Pneumology (n57) and Internal Medicine (n55). 16 other symptoms are detailed in table 1. anti-PL7 positive patients were identified due to immunologi- cal laboratory databases. To exclude false-positive patients, we 11 patients complained of mild (NYHA II, n55) to severe (NYHA only included patients who successively tested positive for III–IV, n56) dyspnoea as an initial symptom of ILD. Dyspnoea anti-PL7 twice (n513): the three patients tested only once did was chronic (n57) or acute (n54), and could be associated with not disclose any symptoms in accordance with ASS. We dry cough (n57) and clubbing (n52). Examination revealed fine excluded one patient for whom clinical data were missing. The crackles in all patients. ILD was confirmed by high-resolution 12 included patients tested positive for anti-PL7 antibodies at computed tomography (CT) scans in all cases. Based on this CT least twice by immunodot (immuno-DOT D-tek; Diasorin, scan, nine out of 12 patients exhibited nonspecific interstitial Antony, France) and/or Western blot using protein extracts pneumonia (NSIP) pattern with bibasal mild fibrosis, including from Hep2 cells (n57 and n55, respectively). They presented ground-glass and intralobular reticulation. Traction bronchiecta- with one or more ASS symptoms, including ILD and/or sia were initially described in four of these patients. A pattern myositis. A search for anti-DNA and anti-extractable nuclear of organising pneumonia (OP) was present in a further two antigen antibodies was systematically performed, whereas cases, with subpleural and peribronchovascular condensations. rheumatoid factor and anti-citrullinated peptide were available Ground-glass opacities were associated with bronchial wall in four and two patients, respectively. thickening and mosaı¨c pattern in the remaining case, in accordance with obliterative bronchiolitis (OB). The characterisation of the ILD pattern was made by several radiologists who were all experienced in ILD and were based on Bronchoalveolar lavage (BAL) revealed alveolitis with an the American Thoracic Society/European Respiratory Society increased number of neutrophils (five out of eight patients) 2002 consensus [6]. or lymphocytes (three out of eight patients). Lung biopsy was 714 VOLUME 37 NUMBER 3 EUROPEAN RESPIRATORY JOURNAL EUROPEAN RESPIRATORY JOURNAL TABLE 1 Characteristics of the patients Patient 12 3456789101112 Sex/ethnicity M/C F/Af F/C F/C F/C F/C M/C F/C F/C F/Af M/C F/Af Age at diagnosis yrs 71 56 31 71 38 71 41 74 73 52 41 57 Follow-up months 41 45 115 95 3 70 19 4 18 7 13 31 # TLC– DL,CO/AV 109/63 56/26 70/74 79/ND 98/73 73/88 47/41 ND/ND 69/32 75/69 52/53 59/48 Lung involvement NSIP NSIP NSIP OB NSIP NSIP NSIP OP NSIP OP NSIP HRCT scanner NSIP Alveolitis (BAL) % N 36 N 39 ND N 85 L 43 ND N 40 ND ND L 42 L 46 N 36 Muscle involvement No Yes Yes No No No Yes Yes No No Yes Yes Raynaud’s phenomenon No No No No Yes No No No No No No No Fever No Yes Yes No Yes No No Yes Yes Yes Yes Yes VOLUME 37 NUMBER 3 Mechanic’s hands Yes No No No No No Yes Yes No No Yes No Rheumatic symptoms No Yes No No Yes No Yes No Yes No No No Signs of SSc No No Megacap. SSc-l PHT Megacap. GOR No PHT SSc-l No No PHT oesophagus GOR anti-Scl70 oesophagus Signs of SS No Parotidomegally Chisolm III anti- Dry eyes, Dry mouth, No No No No Dry eyes, No SSA-60 dry mouth Chisholm III dry mouth Dry mouth anti-SSA-60 First-line treatment Steroids Steroids Steroids MTX Steroids Steroids None Steroids Steroids Steroids Steroids Steroids Steroids CYC CYC+ I.V. Ig CYC - AZA I.V. Ig Nth-line IS treatment (reason) 0 1 (Muscle) 4 (Muscle) 2 (lung) ND 0 0 0 0 0 0 3 (lung ) Pulmonary evolution Better Stable Stable Worse ND Stable Better Worse, death Better Better Better Worse, death TLC: total lung capacity; DL,CO: diffusing capacity of the lung for carbon monoxide; AV: alveolar ventilation; HRCT: high-resolution computed tomography; BAL: bronchoalveolar lavage; SSc: systemic sclerosis; SS: Sjo¨gren syndrome; IS: immunosuppressive; M: male; F: female; C: Caucasian; Af: African; ND: not determined; NSIP: nonspecific interstitial pneumonia; OB: obliterative bronchiolitis; OP: organising pneumonia; N: neutrophil count; L: lymphocyte count; Megacap.: megacapillaries; SSc-l oesophagus: SSc-like mega-oesophagus; PHT: pulmonary hypertension; GOR: gastro-oesophageal reflux; anti-SSA-60: anti-Ro/SSA-60kDa # 715 antibodies; CYC: cyclophosphamide; Ig: immunoglobulins; MTX: methotrexate; AZA: azathioprine. : DL,CO c performed in only one patient and confirmed a diagnosis Like anti-Jo1 or anti-PL12 ASS [1, 8, 9], this series of anti-PL7 of OP. The initial pulmonary function tests in 11 patients ASS revealed that lung involvement was frequent (100%), (performed during the first 5 months after diagnosis, three whereas muscular involvement (50%) and other symptoms patients being treated by immunosuppressive drugs) disclosed associated with ASS were less common, including Raynaud’s an isolated restrictive syndrome in six cases. The diffusing phenomenon, which was particularly rare in this series (8%). capacity of the lung for carbon monoxide/alveolar volume Although these results should be interpreted carefully since ratio was significantly decreased in eight patients. Only one our study is retrospective, the high prevalence of ILD and low nonreversible obstructive syndrome was encountered at diag- prevalence of other symptoms is in agreement with observa- nosis (the patient with an OB pattern at CT scan). This obstruc- tions from previous anti-PL7 series [2–4]. tive syndrome worsened during the follow-up: the ratio residual volume/total lung capacity became .140% [7]. In some instances, and as previously described for anti-Jo1 ASS [1], anti-PL7 ASS could appear as an overlapping syndrome. Echocardiography revealed pulmonary hypertension at diag- Besides myositis, an overlap between SSc [3] and ASS has been nosis in two patients and during the follow-up in a third patient proposed by others. No signs of skin sclerosis were noted in (pulmonary arterial pressures of 55–70 mmHg). Asymptomatic any of the anti-PL7 ASS cases in this study. Nevertheless, pericarditis was fortuitously diagnosed in four patients. potential signs of SSc were common (58%). However, no patient could be classified as SSc according to the 1980 Muscular involvement occurred mainly at diagnosis (five out American College of Rheumatology classification and only of six patients).
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