Abstract
Pulmonary manifestations can be present in 20-80% of patients having mixed connective tissue disorder (MCTD) and are usually subacute. MCTD when associated with polymyositis can rarely involve the diaphragm, causing respiratory failure.We present herein the case of a 49-year-old female having MCTD with a component of polymyositis who presented with bilateral diaphragmatic paralysis followed by heart failure requiring respiratory support with non-invasive mechanical ventilation. We are aware of only one prior instance of MCTD associated with unilateral diaphragmatic weakness causing mild respiratory dysfunction. To the best of our knowledge, this is the second reported case of diaphragmatic involvement in the MCTD population, with bilateral diaphragmatic paralysis causing severe respiratory failure. This is also the first reported case of such an unusual initial presentation in this patient group. Pulmonary involvement has a poor prognosis. Early diagnosis with the initiation of therapy can improve mortality outcomes in this patient population.
Highlights
The definition of mixed connective tissue disorder (MCTD) is constantly evolving
PSW: positive sharp waves, Fibs: fibrillation, Fasc: fasciculation, CRD: complex repetitive discharges, Amp: amplitude, Dur: duration, N: normal. She was diagnosed with MCTD with components of systemic lupus erythematosus (SLE) and polymyositis based on the combination of her clinical history with her autoimmune panel and biopsy results
MCTD can present with respiratory failure as the initial presenting symptom
Summary
The definition of MCTD is constantly evolving. Currently, the incidence of MCTD is about two persons per 100,000 person-years [1]. A 49-year-old lady with a history of multiple hospital admissions for chronic obstructive pulmonary disease exacerbation (COPD) and hypercarbic respiratory failure requiring to be treated with noninvasive mechanical ventilation (NIV), presents to the clinic for evaluation of an abnormal autoimmune panel. PSW: positive sharp waves, Fibs: fibrillation, Fasc: fasciculation, CRD: complex repetitive discharges, Amp: amplitude, Dur: duration, N: normal She was diagnosed with MCTD with components of systemic lupus erythematosus (SLE) and polymyositis based on the combination of her clinical history with her autoimmune panel and biopsy results. She was started on intravenous immune globulin (IVIg) therapy for four days along with a steroid tapered dose. The patient is scheduled to receive maintenance IVIg treatment every six months
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