¹Afnan Tayeb, ¹Aya Saad, ¹Samah Allam, ¹Noha Yaseen,
¹Al Qassimi Hospital

Background(s):

Cerebritis and pericarditis are rare manifestations of lupus; they present with a wide variety of symptoms ranging from psychiatric manifestation in cerebritis to chest pain in pericarditis. In this article, we present a case of a 15 years old boy known to have systemic lupus erythematosus, lupus nephritis, and sickle cell anemia who was admitted to the hospital with sickle cell crisis; however, his hospital course was complicated with lupus cerebritis and pericarditis as the patient developed pericardial effusion and seizure. Imaging and blood tests were conducted; however, pericardiocentesis was not done due to family refusal. The patient received intravenous steroids, intravenous immunoglobulins, and intravenous cyclophosphamide, after which a marked improvement was witnessed.

Introduction:

Systemic lupus erythematosus is a chronic autoimmune and multisystem disease with various manifestations ranging from fever and joint involvement to organ diseases such as cerebritis and pericarditis. Despite their names, cerebritis ,and pericarditis both can be very challenging to diagnose due to the wide range of symptoms that both can present with. However, they are treatable if highly suspected and were early intervened. Early diagnosis is required to prevent complications. This can include biomarkers, imaging, and tissue biopsies. Treatment includes intravenous steroids and immunoglobulins in addition to chemotherapeutic medications such as cyclophosphamide. We report a case of sickle cell anemia and systemic lupus erythematous with lupus nephritis who presented with sickle cell crisis and developed serositis, mainly pericarditis with Cerebritis.

Method(s):

It is a case of a 15-year-old boy with sickle cell anemia, systemic lupus erythematosus, and lupus nephritis on hydroxychloroquine, tacrolimus, prednisolone, and enalapril as maintenance therapy. Diagnosed with Lupus and Lupus nephritis in November 2017 and had a kidney biopsy which showed grade III lupus nephritis. The second kidney biopsy done in May 2020 due to progressive proteinuria and renal derangement showed focal proliferative and diffuse membranous glomerulonephritis consistent with lupus nephritis class III Plus V with mild activity and mild chronicity. He was started on methylprednisolone pulse dose of 1000 milligrams and six doses of cyclophosphamide between May 2020 and October 2020 as per NIH protocol.

He presented to our hospital in February/2023 with a fever, non-productive cough, abdominal pain, and vomiting. His initial vitals indicated a heart rate of 111 when the patient developed severe chest pain and right shoulder pain later that day. His physical examination initially showed no abnormalities, and a laboratory investigation revealed 5.7 grams/dl of hemoglobin (down from 8 grams/dl one month before the current presentation), hyperkalemia (potassium level 6 ), and mild renal impairment. The initial diagnosis was consistent with a painful sickle cell crisis. He was started on intravenous hydration and blood transfusions.

Results:

This discussion will tackle concept of lupus serositis, especially Lupus cerebritis in addition to pericarditis. Lupus cerebritis can be a very challenging diagnosis, requiring high index of suspicion due to the variety of presenting symptoms. Early presenting features can be confusing. These symptoms can vary from neuro-psychiatric manifestations including depression and anxiety, non-specific features such as headache and seizures to more severe symptoms like significant seizures, strokes, vision problems, dizziness, altered mental status, or psychosis which are seen in 15% of the cases according to Goswami et al(1) who reported two cases of Lupus cerebritis ,as initial manifestation of SLE, both presented with seizures but with unique distinctive non specific features and treated initially with other diagnosis before further deterioration prompt suspicion of connective tissue disease to reach the final diagnosis of SLE complicated with lupus cerebritis. Similarly, another case report by Leitao et al(2) in which the patient presented as initial manifestation of lupus with status epilepticus and respiratory failure for which ventilatory support was needed. In our case, the patient who is also known to have SLE, initially presented with sickle cell crisis after which he developed seizure and underwent prompt investigations in suspicion of Lupus cerebritis.