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Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by multisystemic organ involvement in which the body's immune system attacks itself. The clinical presentation and the course are variable. Common presentation symptoms may include fever, fatigue, discomfort, joint pain, abdominal pain, and sterile skin rashes. 1 In severe cases, it may be cerebrovascular lupus. confusion, lethargy, convulsions, coma, mood changes and even psychosis. It is a serious condition that is difficult to diagnose but can be treated with early intervention. 2,3 The patient in this case not only developed cerebral lupus, but also catatonia secondary to lupus. There are not many cases reporting the neuropsychiatric manifestation of SLE. 4
Case Presentation
The patient was a 16 year old girl in grade 9 who resided with her mother, grandmother, and two siblings. She had no known psychiatric history and her medical history was significant for chronic anemia and benign heart murmur. The patient had a fluctuating chin rest, fatigue, psychomotor retardation, anorexia (with a 16-pound weight loss), dysarthria with difficulty finding a word, slow speech and unsteady gait. She had no significant recent stressors, head trauma or travel. Laboratory tests have been notable for elevated thyroid hormone, mild anemia, leukopenia, elevated lipase, high rate of erythrocyte sedimentation (ESR), and hematuria on the liver. urinary badysis. A chest x-ray and CT scan of his head were unremarkable. Physical examination revealed slowness of speech and cognition, tachycardia with systolic murmur, non-painful cervical lymphadenopathy, splenomegaly, slight tremor in the left hand, and decreased strength in both limbs. higher. The patient was transferred for further care and admitted to hospital medicine, which consulted neurology. At the time, his mentation had improved and no focal result was noted. Neurology recommended continuation of infectious and metabolic studies, with consultation of psychiatry for possible psychogenic etiology.
During the initial badessment by psychiatry, the patient was able to maintain adequate eye contact and engage in conversation with soft, hypophonic speech. Her thought process was linear and logical and she denied suicidal ideation or active homicide and reported no psychosis. His cognition was grossly intact without significant results. Even though the patient was vulnerable to mental illness because of a history of significant verbal abuse, domestic violence at home, and a family history of psychiatric illness, her current symptoms could not only be unheard of. explain primary psychiatric state. Psychiatry recommended to continue testing for the organic cause in relation to the results of the physical examination (tachycardia, fever, non-contending cervical lymphadenopathy and splenomegaly)
The hospitalization of the patient s & # 39; It is complicated when its sympathetic nervous system has become hyperactive. about 150 beats per minute and an average breathing rate of 25 breaths per minute with uncontrolled blood pressure. Cardiology was consulted to help manage this complication and recommended the imaging of his abdomen for a possible pheochromocytoma and renal artery stenosis. Infectious disease (consulted for fever of patient of unknown origin) found no abnormalities in blood and urine cultures; Other tests revealed normocytic anemia, elevated ESR, normal C-reactive protein and continued hematuria. As a result of these laboratory findings, Rheumatology has been consulted for the sake of an autoimmune disease.
Suspicion for SLE was high due to multisystemic involvement (hematologic, nervous, renal, pulmonary and cardiac systems). Further clinical examination revealed a dermatitis punctuated by punctate palmar erythema, fingertip staining by lipstick and erythema of ulceration on the hard palate. Additional tests were positive for antinuclear antibodies (ANA) and anti-DNA, high myoglobin and low complement 3 and 4. Its urinalysis was positive for moderate blood and small proteins ( Table 1 ). The patient met the criteria for an LED diagnosis ( Table 2 ), so that she started taking steroids in high doses with evacuation plans in a few days; However, the patient's mentoring deteriorated and she was unable to follow orders and continue the conversation. Her airways were compromised and she was transferred to the pediatric intensive care unit for her advancing medical needs. Concern for convulsive activity prompted an electroencephalogram and she was given levetiracetam. The patient continued to decompensate, so that a nasogastric tube was placed to facilitate her dysphagia. Additional support measures have been taken to improve functionality through professional, physical and respiratory therapy. The patient had severe neuropsychiatric systemic lupus with active encephalopathy;
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Table 1: Summary of Significant Laboratory Results of the Patient |
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Table 2 : Systemic Lupus International Collaborating Clinics Clbadification Criteria for Systemic Lupus Erythematosus |
Her hospitalization became more complicated when she became catatonic. On examination, the patient presented himself as non-verbal, unable to follow orders, and presenting with hypokinesia, catalepsy, waxy suppleness, negativism, echopraxis. There was no evidence of mannerism, stereotypical movements, agitation or grimace. 10 Psychiatry was reconsulted and lorazepam recommended, but the patient showed only a slight improvement until the dosage was increased. Electroconvulsive therapy was discussed with the patient's mother, who refused treatment. The patient slowly improved during 2 months and was finally released with appropriate follow-up and medication.
Discussion
This case illustrates several of the challenges of diagnosing and treating SLE and its complications. This case was unique in that the patient had a sudden onset of symptoms. Its evolution was rapidly manifested by neuropsychiatric symptoms, which became a challenge as it had no prior diagnosis of LEDs. However, once the LED was included in the differential diagnosis, the diagnosis was made quickly by laboratory tests measuring autoantibodies. 5
Aggressive measures have been taken to manage systemic lupus. She was given intravenous cyclophosphamide and immunoglobulins, and had three cycles of plasma exchange
. This case also illustrates the importance of first excluding the organic causes of the symptoms of a present disease before suggesting the etiology. Psychiatry is often the first to be consulted when a patient has symptoms of the central nervous system (CNS), but if a primary organic condition can be identified and appropriately treated, CNS symptoms can be alleviated, if not completely alleviated , with the appropriate treatment. 6
In this patient, the correct diagnosis was made and she was able to receive aggressive treatment. However, because of the severity of his acute presentation, the insult to his brain was so important that his condition progressed to catatonia. Catatonia is a disease characterized by an abnormality in movement and behavior. There are several etiologies for catatonia, including SLE. The severity of catatonia can be badessed using the Bush-Francis logging scale, which can also be used to monitor improvement. 7.8
References
- Tsokos GC. Systemic lupus erythematosus. N Engl J Med . 2011; 365: 2110-2121. do I:. doi: 10.1056 / NEJMra1100359 [CrossRef]
- Calabrese L, Stern T. Neuropsychiatric manifestation of systemic lupus erythematosus. Psychosomatics . 1995; 36: 344-359. doi: 10.1016 / S0033-3182 (95) 71644-9 [CrossRef]
- Stojanovich, G Zandman-Goddard, Pavlovich S, Sikanich N. Psychiatric manifestation in systemic lupus erythematosus. Autoimmun Review . 2007; (6): 421-426. do I:. doi: 10.1016 / j.autrev.2007.02.007 [CrossRef]
- Ali A, Taj A, Uz-Zehra M. Lupus catatonia in a girl who presented fever and modified sensorium. Pak J Med Sci . 2014; 30 (2): 446-448.
- Zandman-Goddard G, Chapman J, Shoenfeld Y. Autoantibodies involved in neuropsychiatric SLE syndrome and antiphospholipid syndrome. Arthritis Rheum Seminar . 2007; 36 (5): 297-315. do I:. doi: 10.1016 / j.semarthrit.2006.11.003 [CrossRef]
- Bronheim HE, Fulop G, Kunkel EJ, et al. The Academy of Psychosomatic Medicine practices guidelines for psychiatric consultation in the general medical setting. The Academy of Psychosomatic Medicine. Psychosomatics . 1998; 39 (4): S8-3S0. doi: 10.1016 / S0033-3182 (98) 71317-9 [CrossRef]
- Walther S, Strik W. Catatonia. Spectro CNS . 2016 21: 341-348. do I:. doi: 10.1017 / S1092852916000274 [CrossRef]
- Sienaert P, Dhossche DM, Vancampfort D, De Hert M, Gazdag G. A clinical examination of the treatment of catatonia. Psychiatric Front . 2014; 5: 181. Am I. doi: 10.3389 / fpsyt.2014.00181 [CrossRef]
- Kuhn A, Gonsmann B, Anders H, Herzer P, Tenbrock K, Schneider M. Diagnosis and treatment of systemic lupus erythematosus. Dtsch Arztebl Int . 2015; 112 (25): 423-432. doi: 10.3238 / arztebl.2015.0423 [CrossRef].
- Bush G, Fink M, G Petrides, F Dowling, Francis A. Catatonia. I. Rating scale and standardized examination. Acta Psychiatr Scand .1996; 93 (2): 129-136. do I:. doi: 10.1111 / j.1600-0447.1996.tb09814.x [CrossRef]
Summary of significant laboratory results of the patient
| Antinuclear antibodies | Positive [19659036] Anti-DNA Antibody | > 300 IU / ml |
| Complement 3 and 4 | 43 and 7 mg / dl | |
| Anti-Smith Antibody | <0.2 | |
| White Blood Cell Count [19659035] 2.4 3 / mcL | ||
| Number of red blood cells | 2.7 6 / mcL | |
| Hemoglobin | 8 g / dL | |
| Hematocrit [19659035] 26.8% [19659036] Erythrocyte sedimentation rate | 40 mm / h |
Systemic lupus International Collaborating clinics Criteria for clbadification of systemic lupus erythematosus
| Acute Skin Lupus [196590] 57] Antinuclear antibodies | |
| Chronic cutaneous lupus | Anti-DNA |
| Oral or nasal ulcers | A Anti-Nti-Smith antibody |
| Non-permeable alopecia | Antiphospholipid antibodies |
| Arthritis [19659057] Weak Complement (C3, C4, CH50) | |
| Seritis | Absence of Coombs Test in the Presence of Hemolytic Anemia |
| ] Renal Clinical Symptoms | |
| Neurological Clinical Symptoms [19659057] | |
| Hemolytic anemia | |
| Leukopenia | |
| Thrombocytopenia |
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