Complicated course of systemic lupus erythematosus

Overview

Bubeníková E. Complicated course of systemic lupus erythematosus

Systemic lupus erythematosus (SLE) is an autoimmune inflammatory disease characterized by several variable manifestations and frequent multiorgan involvement, occurring predominantly in women of childbearing age. The clinical picture is very variable, from mild skin-joint involvement to a severe, life-threatening course with serious organ damage. Herein mentioned case report represents a patient with more than twenty years of disease duration. In the beginning, the manifestation of the disease was dominated by laboratory changes and symptoms imitating an infectious disease; organ involvement was not proven at first, therefore therapy with glucocorticoids and antimalarials was started. Unfortunately, over the years, many organ manifestations and complications associated with the underlying disease have developed. A few years after diagnosis, skin-joint involvement with Jaccoud arthropathy, and severe osteoporosis dominated, followed by recurrent soft tissue infections and secondary Sjögren's syndrome. A significant worsening of the condition occurred especially after the verification of lupus nephritis and the initiation of cyclophosphamide therapy, during which the patient suffered from serious infectious complications. So far, however, the most serious was an extensive subarachnoid hemorrhage with an initially very uncertain prognosis, most likely caused by long-term anticoagulation therapy administered for secondary antiphospholipid syndrome. Despite significant complications including secondary meningitis and extensive damage from the brainstem to the C7 region with paresis to plegia of the lower limbs, the patient's condition was stabilized, and intensive physiotherapy was subsequently started. Currently, the patient's condition has improved from a neurological point of view, but recurrent infections and significant changes in the blood count (leukopenia, microcytic anemia) persisted. Taking into account the extreme risk of the patient, a cautious treatment strategy was chosen in the form of belimumab therapy, a reduced dose of mycophenolate mofetil, hydroxychloroquine, and long-term higher doses of glucocorticoids. The patient is now stabilized, and with the current therapy, she reports an improvement in her general condition. This case report aims to point out the wide spectrum of possible complications of systemic lupus erythematosus and the necessary role of multidisciplinary cooperation.

Keywords:

Systemic lupus erythematosus – immunosuppressive therapy – antiphospholipid syndrome – subarachnoid hemorrhage – multiorgan complications