Abstract Introduction Melanoma differentiation associated (MDA)-5 is a gene product that aids in antiviral immunity. Autoantibodies against this product can result in clinical manifestation noted by nodules to metacarpophalangeal (MCP) or interphalangeal joints (Gottron papules), arthritis, facial rash, and rapidly progressive interstitial lung disease (RP-ILD). The morbidity and mortality with MDA-5 associated dermatomyositis is largely attributed to RP-ILD. Case The patient is a 60-year-old male with a past medical history of osteoarthritis who presented with a chief complaint of fatigue starting 3 weeks prior to presentation. He endorsed night sweats, weight loss, and persistent cough over the same time period. He is a never smoker with occasional marijuana use, and worked as a painter utilizing masks while working. Physical exam notable for erythema to the nasolabial folds, darkened nailbeds, erythematous/purple discoloration to various MCP joints of bilateral hands with skin ulceration to left third MCP joint, and rales to the right upper lobe lung field. Work up notable for CT chest showing peribronchovascular and peripheral ground glass opacities with reticulation and areas of bronchiectasis with upper and mid lung zone predominance, with mediastinal and right hilar lymphadenopathy. Bronchoscopy with transbronchial biopsy notable for benign reactive bronchial cells with no evidence of malignancy, and bronchoalveolar lavage with lymphocytic predominance. Extensive rheumatologic evaluation performed and pertinent for positive anti-smooth Ab, ANA by IFA with homogenous and speckled pattern, IL-2R, and MyoMarker which was collected and sent out but did not result during hospitalization. The patient’s symptoms improved throughout hospitalization and close follow up with pulmonary medicine and rheumatology was arranged prior to discharge. Discussion A multidisciplinary conference concluded that the patient’s clinical presentation appears most consistent with an organizing pneumonia versus diffuse alveolar damage versus RP-ILD. Repeat chest x-ray showed progressive lung disease and the patient was started on prednisone. Follow up with dermatology was arranged where a shave biopsy was performed which showed lichenoid dermatitis with erythrocyte extravasation. A nonspecific finding but can be seen in dermatomyositis. A few days later, the patient’s MyoMarker panel was weakly positive for anti-MDA5 Ab suggesting an anti-MDA5 dermatomyositis related RP-ILD. Due to fiscal constraints, the patient was unable to stay in the United States for further treatment and had to return to Nicaragua. International care was coordinated with the patient’s cousin and a university hospital in Nicaragua. This case highlights the importance of maintaining a high index of suspicion and rapid treatment for RP-ILD. This abstract is funded by: None
Patel et al. (Fri,) studied this question.