Spontaneous pneumomediastinum in limited cutaneous systemic sclerosis and myositis overlap

Abstract

A 58-year-old man with limited cutaneous systemic sclerosis and myositis overlap complicated by interstitial lung disease presented with several months of progressive dyspnoea and weakness. CT chest revealed extensive pneumomediastinum that was not present on imaging 6 months before this study and appeared to be spontaneous, with no preceding trauma, infection or invasive procedures.

Spontaneous Pneumo-Mediastinum in a Post-COVID-19 Patient with Systemic Sclerosis

“…Spontaneous pneumo-mediastinum has occasionally been reported in patients with SSc (Table 1) [11,[19][20][21], in one case occurring after pulmonary function testing [22]. Most of the patients presented with acute dyspnea [11,[19][20][21], but subacute dyspnea [10] and asymptomatic course [23] are also possible. Pneumo-mediastinum and pneumothorax are often due to the rupture of subpleural cysts [19].…”

“…Most of the patients presented with acute dyspnea [ 11 , 19 , 20 , 21 ], but subacute dyspnea [ 10 ] and asymptomatic course [ 23 ] are also possible. Pneumo-mediastinum and pneumothorax are often due to the rupture of subpleural cysts [ 19 ].…”

“…Pleural irregularities associated with the ILD are frequently described in SSc patients [ 3 ], despite that pleural involvement, including pleural effusion and pneumothorax, are a quite rare complication [ 8 , 9 ]. Pneumothorax and pneumo-mediastinum are ILD complications mainly due to the rupture of subpleural cysts in patients with underlying ILD [ 3 , 10 , 11 ]. Pneumothorax has to be considered a potentially life-threatening complication since re-expansion of the lung is slow, and it is associated with a poorer prognosis [ 11 ].…”

Spontaneous pneumomediastinum developed after steroid pulse therapy in diffuse cutaneous systemic sclerosis patient: A case report

“…The five previous cases described in detail and the present case are summarized in Figure 1h. [1][2][3][4][5] There were no tendencies in subtypes and autoantibody profile. All the patients had ILD.…”

Pneumomediastinum as a Manifestation of Granulomatosis with Polyangiitis

“…This condition can either present spontaneously or secondary to manipulation of the patient's respiratory and digestive systems by trauma, infections, a perforated viscus, or via medical interventions [ 6 ]. Data reporting associations between pneumonediastinum and rheumatological diseases are limited, and this association, however, was more commonly pronounced in patients with concomitant interstitial lung disease, as with inflammatory myopathies, systemic lupus erythematosus and systemic sclerosis [ 7 ]. There was a case report published in 2014 documenting a presentation of pneumomediastinum in a patient with microscopic polyangitis that was preceded by interstitial pneumonia [ 8 ].…”