Chest
Postgraduate Education CornerPulmonary and Critical Care PearlsA 34-Year-Old Man With Amyopathic Dermatomyositis and Rapidly Progressive Dyspnea With Facial Swelling
Section snippets
Physical Examination
The patient's skin demonstrated typical findings of dermatomyositis. His head and neck were swollen with subcutaneous emphysema and crepitance. Diffuse crackles were noted throughout all lung fields. The cardiac examination demonstrated a Hamman sign. Muscle strength was normal.
Laboratory Findings
A hemogram finding and creatine kinase level were normal. The hospital admission chest radiograph and chest CT scan are shown in Figures 2, top and bottom, respectively.
Questions: What relationship exists between the patient's underlying condition and the intrathoracic events? How should this patient be managed?
Diagnosis: Pneumomediastinum and subcutaneous emphysema secondary to amyopathic dermatomyositis-associated interstitial lung disease
Dermatomyositis is a systemic inflammatory disease with onset most commonly in the fifth decade of life. It is characterized by proximal muscle weakness and characteristic cutaneous manifestations that include the following: (1) violaceous rash over extensor aspects of interphalangeal joints, elbows, and knees (Gottron papules); (2) heliotrope discoloration of the eyelids, often with periorbital edema; (3) macular erythema of posterior shoulders and neck; and (4) hyperpigmentation,
Clinical Pearls
ILD is a common complication of dermatomyositis and can occur before cutaneous or muscular manifestations of the disease.
Patients with the NSIP and COP subtypes may respond to glucocorticoid therapy and portend a better prognosis than in patients with DAD or UIP.
Given the high prevalence of ILD in patients with dermatomyositis and the potential benefit of early therapy for subsets of patients with NSIP and COP, some experts have recommended high-resolution chest CT imaging for all patients with
ACKNOWLEDGMENT
The authors thank Richard Sontheimer, MD, for his thoughtful comments, and Chris Siegenthaler, for assistance with the manuscript preparation.
References (0)
Cited by (16)
Spontaneous pneumomediastinum: Analysis of 62 consecutive adult patients
2009, Mayo Clinic ProceedingsCitation Excerpt :The presence of underlying lung disease may adversely affect the outcome of patients with SP. For example, a recent report described a patient with amyopathic dermatomyositis-associated interstitial lung disease who developed tension pneumomediastinum requiring tube drainage and died of acute respiratory distress syndrome.13 Tension pneumomediastinum or tension pneumopericardium results from persistent entry of air into the mediastinal or pericardial spaces via a 1-way valve mechanism, resulting in increasing gas pressure in these compartments.
Pneumomediastinum and diffuse alveolar pain. Severe interstitial pneumopathy due to dermatomyositis
2009, Reumatologia ClinicaThe long-term prognosis of pneumomediastinum associated with dermatomyositis: A two-centre retrospective cohort study
2021, Rheumatology (United Kingdom)Inflammatory Myopathy-Related Interstitial Lung Disease: From Pathophysiology to Treatment
2020, Frontiers in MedicineHoarseness and laryngeal lesions may be poor prognostic factors for pneumomediastinum in dermatomyositis with interstitial lung disease
2017, International Journal of Clinical and Experimental Medicine
The authors have reported to the ACCP that no significant conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.