□
PICTURES IN CLINICAL MEDICINE
□
Periungual Hemorrhage with Rapid Progressive Interstitial Pneumonia Makoto Hibino, Naoki Sawamura, Motoki Ohe and Tetsuri Kondo Key words: amyopathic dermatomyositis, anti-MDA5 antibody, periungual hemorrhage, rapid progressive interstitial pneumonia (Intern Med 54: 2083-2084, 2015) (DOI: 10.2169/internalmedicine.54.4512)
Picture 1.
Picture 2.
A 64-year-old previously healthy Japanese man presented with a 14-day history of nausea, vomiting, diarrhea, and fever (37-38℃), and a 3-day history of dry cough, without arthralgia, myalgia, or muscle weakness. De-saturation, crackles on auscultation, consolidations on chest computed tomography (Picture 1), and an acute onset suggested bacterial pneumonia. However, interstitial pneumonia associated with dermatomyositis could not be ruled out because of the presence of periungual hemorrhage (Picture 2, black arrows) and erythema. On admission, a complete blood count revealed no abnormality. The laboratory findings revealed elevated levels of serum C-reactive protein (4.62 mg/dL), ferritin (718.2 ng/mL), surfactant protein-A (46.3 ng/mL), and
Department of Respiratory Medicine, Shonan Fujisawa Tokushukai Hospital, Japan Received for publication November 12, 2014; Accepted for publication January 13, 2015 Correspondence to Dr. Makoto Hibino, [email protected]
2083
Intern Med 54: 2083-2084, 2015
DOI: 10.2169/internalmedicine.54.4512
Krebs von den Lungen-6 (834 U/mL), and normal levels of serum creatine phosphokinase, aldolase, and surfactant protein-D. Serology tests for several autoantibodies, including anti-nuclear and anti-aminoacyl tRNA synthetase antibodies, were negative. He was treated with steroids, cyclophosphamide, tacrolimus, and broad-spectrum antibiotics; however, he nevertheless died 14 days after admission because of respiratory failure. An autopsy revealed diffuse alveolar damage and perivascular lymphocyte proliferation in the lungs without myositis. Additionally, anti-melanoma differentiation-associated gene 5 (MDA5) antibody was identified in his serum (1). Considering the clinical, pathological, and serological findings, a diagnosis of amyopathic dermatomyositis with rapid progressive interstitial pneumonia was thus made. Periungual hemorrhage reflects the mi-
crovasculopathy and disease activity associated with dermatomyositis (2); therefore, close attention should thus be paid to patients with such a presentation even in cases of suspected community-acquired pneumonia. The authors state that they have no Conflict of Interest (COI).
References 1. Sato S, Hoshino K, Satoh T, et al. RNA helicase encoded by melanoma differentiation-associated gene 5 is a major autoantigen in patients with clinically amyopathic dermatomyositis: Association with rapidly progressive interstitial lung disease. Arthritis Rheum 60: 2193-2200, 2009. 2. Rider LG, Atkinson JC. Gingival and periungual vasculopathy of juvenile dermatomyositis. N Engl J Med 360: e21, 2009.
Ⓒ 2015 The Japanese Society of Internal Medicine http://www.naika.or.jp/imonline/index.html
2084
PICTURES IN CLINICAL MEDICINE
□
Periungual Hemorrhage with Rapid Progressive Interstitial Pneumonia Makoto Hibino, Naoki Sawamura, Motoki Ohe and Tetsuri Kondo Key words: amyopathic dermatomyositis, anti-MDA5 antibody, periungual hemorrhage, rapid progressive interstitial pneumonia (Intern Med 54: 2083-2084, 2015) (DOI: 10.2169/internalmedicine.54.4512)
Picture 1.
Picture 2.
A 64-year-old previously healthy Japanese man presented with a 14-day history of nausea, vomiting, diarrhea, and fever (37-38℃), and a 3-day history of dry cough, without arthralgia, myalgia, or muscle weakness. De-saturation, crackles on auscultation, consolidations on chest computed tomography (Picture 1), and an acute onset suggested bacterial pneumonia. However, interstitial pneumonia associated with dermatomyositis could not be ruled out because of the presence of periungual hemorrhage (Picture 2, black arrows) and erythema. On admission, a complete blood count revealed no abnormality. The laboratory findings revealed elevated levels of serum C-reactive protein (4.62 mg/dL), ferritin (718.2 ng/mL), surfactant protein-A (46.3 ng/mL), and
Department of Respiratory Medicine, Shonan Fujisawa Tokushukai Hospital, Japan Received for publication November 12, 2014; Accepted for publication January 13, 2015 Correspondence to Dr. Makoto Hibino, [email protected]
2083
Intern Med 54: 2083-2084, 2015
DOI: 10.2169/internalmedicine.54.4512
Krebs von den Lungen-6 (834 U/mL), and normal levels of serum creatine phosphokinase, aldolase, and surfactant protein-D. Serology tests for several autoantibodies, including anti-nuclear and anti-aminoacyl tRNA synthetase antibodies, were negative. He was treated with steroids, cyclophosphamide, tacrolimus, and broad-spectrum antibiotics; however, he nevertheless died 14 days after admission because of respiratory failure. An autopsy revealed diffuse alveolar damage and perivascular lymphocyte proliferation in the lungs without myositis. Additionally, anti-melanoma differentiation-associated gene 5 (MDA5) antibody was identified in his serum (1). Considering the clinical, pathological, and serological findings, a diagnosis of amyopathic dermatomyositis with rapid progressive interstitial pneumonia was thus made. Periungual hemorrhage reflects the mi-
crovasculopathy and disease activity associated with dermatomyositis (2); therefore, close attention should thus be paid to patients with such a presentation even in cases of suspected community-acquired pneumonia. The authors state that they have no Conflict of Interest (COI).
References 1. Sato S, Hoshino K, Satoh T, et al. RNA helicase encoded by melanoma differentiation-associated gene 5 is a major autoantigen in patients with clinically amyopathic dermatomyositis: Association with rapidly progressive interstitial lung disease. Arthritis Rheum 60: 2193-2200, 2009. 2. Rider LG, Atkinson JC. Gingival and periungual vasculopathy of juvenile dermatomyositis. N Engl J Med 360: e21, 2009.
Ⓒ 2015 The Japanese Society of Internal Medicine http://www.naika.or.jp/imonline/index.html
2084
