A 39‐year‐old woman with Lupus, Myositis, and a Recalcitrant Vasculopathy

Abstract

History of the present illness The patient had been given the diagnosis of systemic lupus erythematosus (SLE) 10 years before presentation when she developed a malar rash, Raynaud’s phenomenon, arthralgias, alopecia, and sicca symptoms. She was treated with hydroxychloroquine alone until 6 months before presentation, when she developed digital ulcerations and leukopenia. Hydroxychloroquine was discontinued by her primary rheumatologist and prednisone 60 mg daily was initiated. Over the next 3 months, the patient was admitted to an outside hospital several times and treated with intravenous methylprednisolone for recurrent hand vasculopathy, digital ulcers, and progressive anemia. Three months before presentation to our center, the patient developed intermittent fevers and severe proximal lower extremity myalgias associated with weakness. Her hand lesions were only partially responsive to intravenous glucocorticoids, and the lower extremity pain and weakness persisted despite this therapy. Extensive evaluation of her fevers, including routine blood, urine, and sputum cultures as well as viral and fungal serologies, determined no infectious etiology. Additional evaluation included computed tomography of the chest, which demonstrated scattered bilateral pulmonary nodules measuring up to 8 mm in size. Malaria smears were negative. A tuberculin skin test was negative, and a QuantiFERON assay (Cellestis Limited) was indeterminate. Acid-fast and fungal stains from induced sputum were negative. The patient was treated empirically with broadspectrum antibiotics, but her symptoms persisted. Thoracolumbar magnetic resonance imaging (MRI) was performed to evaluate her lower extremity pain and weakness. There was no evidence of transverse myelitis, paravertebral abscess, or malignancy. Muscle enzymes, including creatine kinase (CK), aldolase, aspartate and alanine aminotransferase, and lactate dehydrogenase (LDH) levels, were within normal limits. However, an MRI study of the thighs displayed scattered enhancement in the bilateral quadriceps. This was believed to be consistent with inflammatory myositis. The patient was transferred for further evaluation and management. Upon transfer, the patient reported severe fatigue, diffuse myalgias that primarily affected the thighs, and pain and swelling in the joints of her hands and knees. She noted some healing of her hand ulcerations, but patchy cyanosis of her fingertips persisted. She denied any other skin lesions or photosensitivity. She reported weakness in both legs but felt that the strength in her upper extremities and neck was normal. She denied dysphagia, dysphonia, and dyspnea.