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• W2998849090 abstract "Polymyositis and dermatomyositis are idiopathic inflammatory myopathies.1 Various antibodies have been identified as associated with polymyositis/dermatomyositis, and characteristic phenotypes have been reported for each.2, 3 Anti-melanoma differentiation-associated gene 5 (MDA-5) antibody is associated with rapid progressive interstitial pneumonia, and cases with high ferritin are reported to have an extremely poor prognosis.4, 5 We report a case of simultaneous onset of anti-MDA-5 antibody-positive dermatomyositis accompanied by interstitial pneumonia and pulmonary tuberculosis. A 43-year-old man developed anti-MDA-5 antibody-positive dermatomyositis accompanied by interstitial pneumonia and pulmonary tuberculosis. Simultaneous immunosuppressive therapy and anti-tuberculosis therapy produced a good outcome. He developed erythema on his face as well as muscle pain and weakness of the limbs, especially his lower limbs, in September 2017. He was admitted to hospital because from December 2017 he developed palpitations and dyspnea during exertion. Physical examination demonstrated a body temperature of 38°C, fine crackles in the lower lung fields on both sides, Gottron's papules, and an erythematous rash on his face. Muscle pain in the proximal limb muscles and muscle weakness of about 4/5 on the Manual Muscle Test were confirmed. Laboratory examinations showed creatine kinase (CK) 76 U/L (normal range: 59-248 U/L), aldolase (ALD) 16.8 U/L (normal range: 2.1-6.1 U/L), sialylated carbohydrate antigen Krebs von den Lungen-6 (KL-6) 700 U/mL (normal range: <500 U/mL), ferritin 1337 ng/mL (normal range: 40-360 ng/mL), C-reactive protein (CRP) 0.32 mg/dL (normal range: <0.3 U/mL), and high titer of anti-MDA-5 antibody (>150) (normal range: <32). Arterial blood gas analysis revealed a PaO2 of 77.9 mm Hg in room air. Chest computed tomography (CT) revealed a cavity on the left S1 + 2 and consolidations and ground-glass opacities in the bilateral lower lung fields, suggesting a radiologic pattern of non-specific interstitial pneumonia (Figure 1). Because we suspected pulmonary tuberculosis from the cavity, we performed a sputum examination. It was positive by acid-fast bacilli sputum smear and positive by tuberculosis-polymerase chain reaction (Tb-PCR). Based on the above, he was diagnosed as dermatomyositis with interstitial pneumonia coexisting with active pulmonary tuberculosis. On 28 January 2018, he was started on anti-tuberculosis therapy consisting of isoniazid (INH), rifabutin (RBT), ethambutol (EB) and pyrazinamide (PZA). After confirming there were no side effects related to these drugs, prednisolone (PSL) at a dose of 60 mg (1 mg/kg) daily and tacrolimus (TAC), the blood trough concentration of which was adjusted to 5-10 ng/mL, were orally administered. These treatments had an antipyretic effect and improved muscle pain and muscle weakness. After anti-MDA-5 antibody positivity was observed, intravenous pulse cyclophosphamide (IVCYC) 500 mg/m2 weekly was initiated. The cavity shrank 1 month after the start of treatment and interstitial pneumonia in the bilateral lower lobes was improved. EB and PZA were discontinued on day 60 as the results of drug susceptibility studies demonstrated that the isolate was susceptible to first-line agents. Because his serum ferritin was still high after 6 cycles of IVCYC, high-dose IV immunoglobulin therapy (IVIg) 20 g daily for 5 days was initiated. We confirmed consecutive negative sputum smears for acid-fast bacillus and improved consolidations and ground-glass opacities on chest CT (Figure 1); therefore, he was discharged on day 95 of hospitalization. Thereafter, there was no exacerbation as an outpatient even though PSL was tapered to 25 mg daily. Furthermore, the serum levels of ferritin and anti-MDA-5-antibody decreased to 548 ng/mL and 117 indexes, respectively (Figure 2). One year later, tuberculosis treatment was completed and PSL was tapered to 7.5 mg daily. Anti-MDA-5-antibody decreased by 31 indexes. To the best of our knowledge, this is the first clinical report of a case of simultaneous onset of anti-MDA-5 antibody-positive dermatomyositis accompanied by interstitial pneumonia and pulmonary tuberculosis. Polymyositis and dermatomyositis are chronic inflammatory disorders that mainly involve muscle and skin lesions. Their clinical features are heterogeneous, with varying degrees of skin manifestations, myositis, and pulmonary involvement. In anti-MDA-5 antibody-positive dermatomyositis patients, rapidly progressive interstitial pneumonia sometimes develops; this condition is resistant to immunosuppressive treatment and is associated with poor outcomes.4, 5 Immediate and intensive immunosuppressive therapy was required in this patient to avoid a fatal situation and the concomitant use of corticosteroid, calcineurin inhibitor, and cyclophosphamide was indispensable to prevent the progression of interstitial lung disease in anti-MDA-5 antibody-positive dermatomyositis.6 Because the effectiveness of IVIg was also reported, it was added to the treatment.7 Subsequently, his skin and muscle symptoms, and interstitial pneumonia improved. Although this patient initially had a high titer of anti-MDA-5 antibody (>upper measurable limit) and serum ferritin exceeding 1000 ng/mL, these were reduced by combination therapy. High serum ferritin levels on admission in anti-MDA-5 antibody-positive dermatomyositis patients were reported to be a poor prognostic marker.8 Whether anti-MDA-5 antibody titers are a prognostic factor is controversial, but previous studies reported that anti-MDA-5 antibody titer was useful for the evaluation of the response to treatment and the status of interstitial pneumonia in patients with anti-MDA-5 antibody-positive dermatomyositis.9 Of note, the reduction of high serum ferritin and anti-MDA-5 antibody levels after treatment indicated they can predict a successful outcome.5, 10 There has been no reported case of simultaneous onset of anti-MDA-5 antibody-positive dermatomyositis and pulmonary tuberculosis. The relationship between dermatomyositis and tuberculosis is unknown. In this case, tuberculosis infection may have triggered the exacerbation of interstitial pneumonia. It is important to report our experience of a patient with anti-MDA-5 antibody-positive dermatomyositis complicated with interstitial pneumonia with high serum ferritin levels and who, despite the complication of infectious disease, obtained a good outcome after combined treatment. Collagen diseases often accompany lung lesions. Thus, this case highlights that attention should be paid to the coexistence of pulmonary tuberculosis. The authors have declared no conflicts of interest. Shota Fujimoto wrote the initial draft of the manuscript. Keisuke Saito and Kazuyoshi Kuwano assisted in the preparation of the manuscript. All authors approved the final version of the manuscript. No specific funding was received from any bodies in the public, commercial or not-for-profit sectors to carry out the work described in this manuscript." @default.
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• W2998849090 title "A case of simultaneous onset of anti‐melanoma differentiation‐associated gene 5 antibody‐positive dermatomyositis accompanied by interstitial pneumonia and pulmonary tuberculosis" @default.
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• W2998849090 doi "https://doi.org/10.1111/1756-185x.13788" @default.
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