case report Wien Klin Wochenschr DOI 10.1007/s00508-015-0823-6
Life-threatening acute pneumonitis in mixed connective tissue disease: a case report and literature review Eva Rath · Shahin Zandieh · Alexander Löckinger · Mirko Hirschl · Klaus Klaushofer · Jochen Zwerina
Received: 12 April 2015 / Accepted: 28 May 2015 © Springer-Verlag Wien 2015
Summary Mixed connective tissue disease (MCTD) is a rare connective tissue disease frequently involving the lungs. The main characteristic is a systemic sclerosislike picture of slowly progressing interstitial lung disease consistent with lung fibrosis, while pulmonary arterial hypertension is rare. Herein, we present a case of a newly diagnosed MCTD patient developing life-threatening acute pneumonitis similar to lupus pneumonitis. Previous literature on this exceptionally rare complication of MCTD is reviewed and differential diagnosis and management discussed.
resenting mainly an overlap between systemic lupus erythematosus (SLE) and systemic sclerosis (SSc). Pulmonary involvement is frequent in MCTD, but the main characteristic is a SSc-like picture of slowly progressing interstitial lung disease (ILD) consistent with lung fibrosis and/or pulmonary arterial hypertension (PAH) [1]. Additionally, pleural effusions due to serositis may occur. However, patients rarely develop acute respiratory failure due to aggressive pulmonary inflammation similar to lupus pneumonitis.
Keywords Mixed connective tissue disease · Pneumonitis · Acute respiratory failure
Case report
Introduction Mixed connective tissue disease (MCTD) is a rare autoimmune disorder defined by the presence of high titer U1RNP autoantibodies and organ manifestations repE. Rath () · K. Klaushofer · J. Zwerina Ludwig Boltzmann Institute of Osteology at the Hanusch Hospital of WGKK and AUVA Trauma Centre Meidling, 1st Medical Department, Hanusch Hospital, Heinrich-Collin-Straße 30, 1140 Vienna, Austria e-mail: [email protected] S. Zandieh Institute of Radiology and Nuclear Medicine, Hanusch Hospital, Vienna, Austria A. Löckinger Institute of Anesthesiology, Hanusch Hospital, Vienna, Austria M. Hirschl Department of Angiology, Hanusch Hospital, Vienna, Austria
13
A 26-year-old woman presented to the rheumatology department with progressive Raynaud’s phenomenon, arthralgia and short episodes of fever for about 6 months. The woman had no previous medical history. Physical examination was unremarkable except elevated body temperature levels of 38.5 °C, diffuse swollen hands, Raynaud’s phenomenon, livid discoloration of the second toe of the right foot, and asymmetric cervical lymphadenopathy. Ultrasound evaluation of the abdomen and lymph nodes revealed reactive lymphadenopathy and mild splenomegaly. Chest X-ray and transthoracic echocardiography were unremarkable. Results of laboratory investigations at admission are shown in Table 1. Videocinematography of the esophagus revealed esophageal dysmotility and nailfold capillary microscopy showed megacapillaries, avascular fields, and thrombotic capillaries. A presumptive diagnosis of MCTD was made but recurrent fever prompted further microbiological workup that remained negative for bacterial, viral, and parasitic infections while the interferon-γ release assay was positive. The patient continued to have fever up to 39 °C and dry cough despite antibiotic therapy with intravenous cefuroxime, which was switched to moxifloxacin after 3 days. Considering the continuous fever as part of
Life-threatening acute pneumonitis in mixed connective tissue disease: a case report and literature review
1
case report
Table 1 Laboratory data Variable
Reference range
At admission
Hemoglobin (g/dl)
12–16
10.9
White blood cell count (x 10 /µl)
4–10
4.7
Platelet count (x 103/µl)
140–400
274
C-reactive protein (mg/l)
0–5
61
Creatinine (mg/dl)
Life-threatening acute pneumonitis in mixed connective tissue disease: a case report and literature review Eva Rath · Shahin Zandieh · Alexander Löckinger · Mirko Hirschl · Klaus Klaushofer · Jochen Zwerina
Received: 12 April 2015 / Accepted: 28 May 2015 © Springer-Verlag Wien 2015
Summary Mixed connective tissue disease (MCTD) is a rare connective tissue disease frequently involving the lungs. The main characteristic is a systemic sclerosislike picture of slowly progressing interstitial lung disease consistent with lung fibrosis, while pulmonary arterial hypertension is rare. Herein, we present a case of a newly diagnosed MCTD patient developing life-threatening acute pneumonitis similar to lupus pneumonitis. Previous literature on this exceptionally rare complication of MCTD is reviewed and differential diagnosis and management discussed.
resenting mainly an overlap between systemic lupus erythematosus (SLE) and systemic sclerosis (SSc). Pulmonary involvement is frequent in MCTD, but the main characteristic is a SSc-like picture of slowly progressing interstitial lung disease (ILD) consistent with lung fibrosis and/or pulmonary arterial hypertension (PAH) [1]. Additionally, pleural effusions due to serositis may occur. However, patients rarely develop acute respiratory failure due to aggressive pulmonary inflammation similar to lupus pneumonitis.
Keywords Mixed connective tissue disease · Pneumonitis · Acute respiratory failure
Case report
Introduction Mixed connective tissue disease (MCTD) is a rare autoimmune disorder defined by the presence of high titer U1RNP autoantibodies and organ manifestations repE. Rath () · K. Klaushofer · J. Zwerina Ludwig Boltzmann Institute of Osteology at the Hanusch Hospital of WGKK and AUVA Trauma Centre Meidling, 1st Medical Department, Hanusch Hospital, Heinrich-Collin-Straße 30, 1140 Vienna, Austria e-mail: [email protected] S. Zandieh Institute of Radiology and Nuclear Medicine, Hanusch Hospital, Vienna, Austria A. Löckinger Institute of Anesthesiology, Hanusch Hospital, Vienna, Austria M. Hirschl Department of Angiology, Hanusch Hospital, Vienna, Austria
13
A 26-year-old woman presented to the rheumatology department with progressive Raynaud’s phenomenon, arthralgia and short episodes of fever for about 6 months. The woman had no previous medical history. Physical examination was unremarkable except elevated body temperature levels of 38.5 °C, diffuse swollen hands, Raynaud’s phenomenon, livid discoloration of the second toe of the right foot, and asymmetric cervical lymphadenopathy. Ultrasound evaluation of the abdomen and lymph nodes revealed reactive lymphadenopathy and mild splenomegaly. Chest X-ray and transthoracic echocardiography were unremarkable. Results of laboratory investigations at admission are shown in Table 1. Videocinematography of the esophagus revealed esophageal dysmotility and nailfold capillary microscopy showed megacapillaries, avascular fields, and thrombotic capillaries. A presumptive diagnosis of MCTD was made but recurrent fever prompted further microbiological workup that remained negative for bacterial, viral, and parasitic infections while the interferon-γ release assay was positive. The patient continued to have fever up to 39 °C and dry cough despite antibiotic therapy with intravenous cefuroxime, which was switched to moxifloxacin after 3 days. Considering the continuous fever as part of
Life-threatening acute pneumonitis in mixed connective tissue disease: a case report and literature review
1
case report
Table 1 Laboratory data Variable
Reference range
At admission
Hemoglobin (g/dl)
12–16
10.9
White blood cell count (x 10 /µl)
4–10
4.7
Platelet count (x 103/µl)
140–400
274
C-reactive protein (mg/l)
0–5
61
Creatinine (mg/dl)
