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MDA-5 associated rapidly progressive interstitial lung disease with recurrent
Pneumothoraces: a case report
#MMPMID29665800
Alqatari S
; Riddell P
; Harney S
; Henry M
; Murphy G
BMC Pulm Med
2018[Apr]; 18
(1
): 59
PMID29665800
show ga
BACKGROUND: Clinically hypomyopathic dermatomyositis is a rare disease that is
important to recognize, investigate and treat early as it is associated with poor
prognosis. In a proportion of patients, myositis specific antibodies could be
negative, but with high clinical suspicion, myositis associated antibodies should
be ordered. Anti-MDA-5 antibodies was reported in literature to be associated
with severe and rapidly progressive interstitial lung disease, with few case
reports of pneumothorax and/or pneumomediastinum. CASE PRESENTATION: A
49-year-old previously healthy lady, presented with a 6 week history of skin
rash, photosensitivity, mouth ulcers, fatiguability, arthralgia and myalgia. She
denied subjective weakness, respiratory symptoms or dysphagia. She had Raynaud's
phenomenon affecting her fingers only. Initial examination showed synovitis in
her hands with skin rash. Autoimmune screen was negative. She was started on
hydroxychloroquine. 4 weeks later on follow-up, she developed proximal muscle
pain, dysphagia, dyspnea and dry cough. Examination showed mild proximal muscle
weakness and bi-basal crackles. She was admitted and extended myositis screen was
sent. She had mild anemia, lymphopenia and neutropenia, normal inflammatory
markers, liver and renal panels. Capillaroscopy showed pattern of systemic
sclerosis. CT chest showed early ILD. Electromyography and MRI showed features of
mild myositis. PFT showed muscle weakness with low DLCO. She was given
intravenous steroid and Rituximab. As she continued to deteriorate, intravenous
immunoglobulins and cyclophosphamide were given. There was a brief clinical
response that was short-lived with increasing oxygen dependency necessitating
transfer to the ICU. At this point, the extended myositis screen confirmed the
presence of anti-MDA-5 antibodies. She commenced plasmapharesis and required
intubation. Unfortunately, she developed multiple pneumothoraces, and was
transferred urgently for ECMO. Subsequent immunosuppression included rituximab
and tacrolimus. There was progression of her ILD and recurrent pneumothoraces and
pneumomediastinum. Unfortunately, she passed away as a consequence of her
disease. CONCLUSION: This case highlights a number of considerations in
approaching patients with inflammatory myositis, particularly to pulmonary
involvement. It is important to highlight the utility of extended myositis
antibody testing in predicting disease phenotypes and its impact on therapeutic
decisions. From a management perspective, aggressive immunosuppression should be
considered with potential need of earlier utilization of ECMO.
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