Clinically amyopathic dermatomyositis with rapidly progressive interstitial pneumonia: The relation between the disease activity and the serum interleukin‐6 level

Abstract

A 67-year-old woman exhibited chilblain-like erythema on the bilateral auricles, erythema with hyperkeratosis on the sides of fingers and the bilateral elbows, and erythema on the extensor/flexor sides of the finger joints and the iliac regions. Fine crackles were audible on the dorsal side of the bilateral lower lung regions. We diagnosed clinically amyopathic dermatomyositis accompanied by rapidly progressive interstitial pneumonia. While we initiated immunosuppressive therapy, the respiratory failure showed no responses. We performed endotoxin adsorption therapy using polymyxin B-immobilized fiber column direct hemoperfusion (PMX-DHP). After the first PMX-DHP session, the respiratory condition was slightly improved but it soon deteriorated. At the second PMX-DHP session, subcutaneous and mediastinal emphysema was noted. The patient died, showing no improvement in the respiratory condition. We measured the levels of serum interleukin (IL)-6, tumor necrosis factor (TNF)-α and IL-10 before and after PMX-DHP and compared them with previously reported levels. The levels of TNF-α and IL-10 showed no or slight changes, but those of IL-6 at the beginning of the treatment were higher compared with previous reports. After the first PMX-DHP, the IL-6 level decreased with slight improvement in respiratory condition. However, after the second PMX-DHP, it increased markedly with exacerbation of the respiratory failure. The serum IL-6 level is considered to be effective for the evaluation of the disease activity and prognosis under the treatment by PMX-DHP.

Relevance of interferon-gamma in pathogenesis of life-threatening rapidly progressive interstitial lung disease in patients with dermatomyositis

“…Serum IFN-γ was characteristically high and correlated with the G-scores in the DM RP-ILD group, whereas serum IL-6 was not elevated characteristically and did not correlate with the CT scores (Fig. 2 and Table 2 ), although IL-6 is reported to be important in DM RP-ILD [ 17 , 19 , 36 , 37 ]. In the next step, we examined the roles of IFN-γ and IL-6 in the pulmonary pathophysiology of DM RP-ILD by immunostaining and hematoxylin-eosin (H&E) staining of lung tissues, hilar lymph nodes, and spleen tissues from two patients from whom specimens were obtained on autopsy (Fig.…”

Multicenter Prospective Study of the Efficacy and Safety of Combined Immunosuppressive Therapy With High‐Dose Glucocorticoid, Tacrolimus, and Cyclophosphamide in Interstitial Lung Diseases Accompanied by Anti–Melanoma Differentiation–Associated Gene 5–Positive Dermatomyositis

“…Notably, recent case reports have indicated the efficacy of plasmapheresis in patients with anti–MDA‐5–positive rapidly progressive ILD . Polymyxin B–immobilized fiber column direct hemoperfusion has also been suggested as potentially effective for ILD in CADM or anti–MDA‐5–positive rapidly progressive ILD , although the effect was controversial . In the future, prospective studies comparing patients who undergo plasmapheresis and those who do not are necessary.…”

COVID-19-associated coagulopathy and disseminated intravascular coagulation

“…However, for ARDS caused by cytokine storms in influenza 2009 (N1H1), respiratory status reportedly improved dramatically after only 4 h of PMX-DHP treatment [ 93 ]. PMX-DHP could also improve respiratory failure with decreased IL-6 in patients with amyopathic dermatomyositis who presented with respiratory failure due to acute progressive interstitial pneumonia [ 94 ]. In addition, the clinical experience of PMX-DHP for ARDS in COVID-19 has been reported in the online journal of the Japanese Society of Infectious Diseases.…”