COPA syndrome can be defined as a complex disorder of the immune system, both at the innate (autoinflammatory) and adaptive (autoimmune) levels (19, 38, 46).COPA codes for the coatomer subunit alpha protein, a part of the coat protein complex I (COPI), which, as a component of the cell’s vesicular transport mechanism, is responsible for the retrograde movement of proteins from the Golgi apparatus back to the endoplasmic reticulum (ER) (19). Here, COPA is linked to autoimmune interstitial lung disease-arthritis syndrome.