Introduction
Systemic Lupus Erythematosus (SLE) is a common autoimmune disease involving multiple organs and affecting all body parts. Being autoimmune means the disease is self-triggering in nature, caused by one's immune system attacking the body's healthy living cells and tissues. SLE is predominantly a women's disease, reported to occur highly in women of reproductive age range from 15 to 44 years. However, its incidence in men is non-negligible as 1 out of 10 people affected by SLE identify as male. Clinical manifestations of SLE are noticed in the majority of the body's organ systems, namely the cardiovascular, hematological, renal, ocular, and pulmonary systems. This article reviews the effects of SLE on the neurological systems of the body - the central nervous system (CNS) and peripheral nervous system (PNS). The neuropsychiatric complications of lupus are extremely diverse and vary greatly between two individuals with the same disease.
What Is Neuropsychiatric SLE?
Neuropsychiatric Systemic Lupus Erythematosus (NPSLE) is defined as lupus that shows clinical manifestations in the nervous system of the body. Also referred to as central nervous system lupus, this disease includes a variety of nervous disorders that may present as mildly symptomatic or be severely debilitating in nature.
What Causes NPSLE?
Not much data has been proclaimed to state the exact reasons for the incidence of NPSLE.
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The presence of antibodies in the serum and cerebrospinal fluid (CSF) is one of the prominent biomarkers to signify the nervous system manifestations of SLE.
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Another factor that may contribute to the neurological symptoms is the increased production of Cytokines from the central or peripheral nervous system.
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Meanwhile, another theory proposes that the autoantibodies that are responsible for SLE pass through a weakened blood-brain barrier system - due to the effects of SLE - leading to inflammatory actions.
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The administration of corticosteroids and adverse effects of certain medications have also been reported to bring about psychiatric manifestations.
How to Diagnose NPSLE?
The diagnosis of NPSLE poses a challenge to clinicians owing to the discrepancy of whether the neuropsychiatric symptoms are associated with lupus itself or are just an independent-borne condition. For an accurate diagnosis to be made, a thorough neurological and psychiatric evaluation must be performed by an expert doctor. neuroimaging studies come in handy to confirm the diagnosis further. The neuropsychiatric symptoms can present themselves at any point of the disease in lupus-affected patients. The treatment protocol to manage the NPSLE is highly individualized based on the severity of the symptoms. However, the prognosis of the disease is fairly poor. The neurological and psychiatric complications account for the highest mortality rate in SLE patients.
What Are the Clinical Manifestations of NPSLE?
The clinical manifestations of SLE are subdivided into two categories for a better understanding of the diagnosis and management of each. There are about 12 syndromes that affect the central nervous system (CNS) and 7 syndromes that are associated with the peripheral nervous system (PNS). Syndromes associated with the CNS are further categorized into focal and diffuse syndromes based on their ability to present the symptoms.
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The focal syndromes include cerebrovascular disease, demyelinating syndrome, headache, aseptic meningitis, movement disorder, myelopathy, and seizures.
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The diffuse syndromes include cognitive dysfunction, mood disorders, anxiety, acute confusional state, and psychosis.
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The syndromes that impact the PNS include guillain-barre syndrome, myasthenia gravis, autonomic disorder, cranial neuropathy, plexopathy, mono, and polyneuropathy.
The neuropsychiatric manifestations are one of the earliest symptoms of systemic lupus erythematosus. The most common of all psychiatric symptoms are depression, anxiety disorder, and cognitive dysfunction.
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Depression is encountered as the most common mood disorder associated with SLE patients. About 65 percent of SLE patients are reported to be clinically depressed. Manic episodes are relatively less frequent. The pathophysiology behind depression is much more related to the adverse effects of administering high-dose Prednisone ( >20 mg ) than the disease activity itself. Patients with severe effects of depression are advised to discontinue overuse of Prednisone and consult a mental health specialist for further improvement.
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Anxiety disorder is another highly common symptom of SLE reported in about 40 percent of patients. Various types of conditions come under anxiety disorder, namely obsessive-compulsive disorder (OCD), prominent anxiety, panic disorders, and general anxiety disorders. Proteinuria (excretion of protein via urination) has also been reported in patients of SLE suffering from anxiety disorder.
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Cognitive dysfunction prevalence is found to be high in patients suffering from SLE. About 60 to 80 percent of patients showed significant cognitive impairment in the form of dementia, visual or verbal memory loss, executive dysfunction, and impaired psychomotor speed. Cognitive dysfunction is also found to be evident in SLE patients suffering from antiphospholipid antibody syndrome (APS). The APS syndrome is characterized by one's immune system targeting the killing of the proteins present in one's blood. This leads to the formation of blood clots in arteries and veins.
How Is NPSLE Managed?
The management of NPSLE patients must be considered from a symptomatic point of view. Each individual patient must be viewed as an independent psychiatric patient who must go through a complete psychological evaluation and be assessed for any external stimuli or conditions that may trigger neurological or psychiatric symptoms.
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Antidepressants, antipsychotics, and anti-anxiety medications are prescribed by the doctor based on the symptoms presented by the patient.
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Immunosuppressant therapy may be initiated in patients to keep the exaggerated immunological activity in check and control the inflammatory response.
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Anti-epileptic drugs may be administered in patients suffering from seizure disorder.
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Patients suffering from antiphospholipid antibody syndrome (APS) are prescribed blood thinners or anticoagulants in order to prevent the formation of blood clots that may pose a life-threatening challenge in some scenarios.
Conclusion
Neuropsychiatric systemic lupus erythematosus is a complex disorder that is extremely difficult to diagnose due to limited diagnostic tools. NPSLE accounts for the highest mortality rate in SLE patients and must be managed symptomatically, as no improved treatment protocols have yet been discovered. New research on improving the diagnostic criteria and treatment plan of NPSLE patients is the need of the hour, and one can hope to achieve this stage in the near future.
