LP-206 ANA-negative lupus presenting with segmental hyalinizing vasculitis and valvular heart disease – a rare case report

DescriptionThe presence of antinuclear antibody (ANA) is usually considered a hallmark of Systemic Lupus Erythematosus (SLE). However, a small group of SLE patients had the typical clinical manifestation of SLE with negative ANA tests. Segmental hyalinizing vasculitis is an orphan disease associated with various diseases including SLE. We report a case of a 29-year-old female presented with a painful skin ulcer on both her upper and lower extremities for 2 weeks. She also noted joint pain, fever, and shortness of breath. The symptoms were accompanied by multiple redness and pus-filled skin ulcer on both arms, thighs, and legs. The remainder examination revealed hair loss, pale conjunctiva, crackles in chest examination, ascites, and edema in both feet. There were also redness and tenderness on both hand at Distal Interphalangeal(DIP), Proximal Interphalangeal(PIP), and Metacarpophalangeal(MCP). Laboratory studies showed anemia, elevated erythrocyte sedimentation rate, and positive LE cell. The antinuclear antibody immunofluorescence (ANA IF) test was negative. A skin biopsy revealed segmented hyalinizing vasculitis suitable for Lupus. A transthoracic echocardiogram showed mildly abnormal left ventricular systolic function with an ejection fraction 45%, moderate mitral regurgitation, and mild aortic regurgitation. Chest X-ray showed signs of pulmonary edema and right pleural effusion. The conditions fulfilled the clinical criteria of SLE, and the patient was diagnosed with ANA-negative SLE. The patient underwent treatment with hydroxychloroquine 200 mg twice daily, methylprednisolone 16 mg twice daily, and desoximethasone cream. The patient showed significant clinical improvement and her ulcer completely resolved after 6 months of treatment, and there is no recurrent ulcer.ConclusionsCase report of a 29-year-old woman diagnosed with ANA-negative SLE with segmental hyalinizing vasculitis and valvular heart disease as the main manifestation of SLE. Early recognition and aggressive SLE therapy of this rare subset of SLE disease showed clinical improvement and completely resolved skin ulcers.