Introduction
Systemic lupus erythematosus (SLE) is a chronic, multisystem autoimmune disease that predominantly affects women of childbearing age, with a male-to-female ratio of approximately 1:9. Its etiology is multifactorial, involving genetic, hormonal, and immunological factors. The clinical spectrum ranges from mild symptoms such as fatigue and rash to severe, potentially life-threatening organ involvement. Commonly affected systems include the skin, joints, kidneys, cardiovascular system, and central nervous system, with lupus nephritis and neuropsychiatric SLE (NPSLE) being among the most serious complications contributing to significant morbidity and mortality. Diagnosis relies on characteristic clinical features supported by serologic markers such as antinuclear antibody (ANA), anti-double-stranded DNA (anti-dsDNA), and low complement levels. While single-organ flares are well documented, simultaneous multiorgan flares are rare and pose significant diagnostic and therapeutic challenges.
This case illustrates such a scenario, with concurrent involvement of the kidneys (lupus nephritis), central nervous system (probable CNS vasculitis and posterior reversible encephalopathy syndrome) and heart (myopericarditis). The case emphasizes the need for early recognition of multisystem flares in SLE and highlights the importance of aggressive, targeted treatment strategies to prevent complications and optimize patient outcomes.
Case Report
A 38-year-old female, a known case of Systemic Lupus Erythematosus (SLE) with recent history of urinary tract infection, lower respiratory tract infections and no known drug allergy, presented to our hospital with complaints of cough with scanty expectoration and breathlessness for the past 2 months. She also reported bilateral lower limb swelling of 2 months duration. Additionally, she had pleuritic chest pain for 1 month and complained of lower back and neck pain with restriction of movements since 06/10/2022. She noted frothy and dark-colored urine. There were no episodes of vomiting, syncope, or visual disturbances.
The patient was on treatment at an outside hospital, where investigations revealed elevated white blood cell count and serum creatinine, prompting referral to our facility for further management. She also had a history of generalized lymphadenopathy, for which an axillary lymph node biopsy was done 1 month ago and was reported as reactive lymphadenitis. She had a recent urinary tract infection treated with antibiotics and a short course of anti-tubercular therapy (ATT) for the neck node swelling, which was subsequently stopped in view of reactive lymphadenitis within 10 days of initiation.
On examination, she was conscious, oriented, and afebrile with bilateral pedal edema. Her vital signs were: pulse rate 110/min, blood pressure 160/120 mmHg, respiratory rate 22/min, oxygen saturation 99% on room air, and capillary blood glucose 120 mg/dl. Respiratory system examination revealed bilateral basal end-inspiratory crepitations. Cardiovascular examination revealed normal S1 and S2 heart sounds. Abdomen was soft with bowel sounds present and hypochondrial tenderness. Central nervous system examination showed no focal neurological deficits.
She was admitted and was started on IV Antibiotics and other supportive measures. During her hospital stay, she experienced a generalized tonic-clonic seizure on day 2 of admission at around 05:00 AM and was shifted to the ICU for further observation and management. MRI Brain showed bilateral symmetric FLAIR hyperintensities in the parieto-occipital regions, suggestive of Posterior Reversible Encephalopathy Syndrome (PRES), with no acute infarcts or macro-hemorrhages. MR angiogram showed multiple suspicious focal tiny areas of narrowing in the posterior and middle cerebral arteries, consistent with probable CNS vasculitis. There was no stenosis or occlusion in the neck arteries.
Echocardiogram revealed concentric left ventricular hypertrophy, Grade II mitral regurgitation, mild global hypokinesia with regional variation, mild left ventricular systolic dysfunction (EF 45–50%), and trace pericardial effusion. Cardiac markers (Troponin-I and NT-proBNP) and CPK-MB were elevated and were suggestive of Myopericarditis. Laboratory investigations showed anemia, leukocytosis, elevated renal parameters, and raised CRP. Complement levels (C3 and C4) were decreased, and Anti-dsDNA antibodies were positive. Urine routine showed pus cells, although both blood and urine cultures were sterile.
Nephrologist opinion was obtained for management of lupus nephritis and hyperkalemia; serial renal function monitoring was done. Neurologist evaluated the patient, and antiepileptics were started. Cardiologist and infectious disease specialist consultations were sought and their management plans were followed. In view of active SLE with nephritis, probable CNS vasculitis, and myopericarditis, the patient was started on intravenous methylprednisolone pulse therapy followed by pulse cyclophosphamide. She was later transitioned to oral steroids at 1 mg/kg along with other supportive medications.
X-ray of the cervical spine was unremarkable. MRI whole spine screening done revealed diffuse disc bulges at L4-L5 and L5-S1 levels causing mild spinal canal and bilateral sac neural indentation, without significant foraminal or cord compression. Cervical MRI showed prominent epidural fat at C3-C4 and C4-C5 levels without thecal sac compromise. Spine surgeon opinion was obtained and conservative management was advised.
She was transfused with one unit of packed red cells. Physiotherapy with interferential therapy (IFT) was initiated. The patient improved with the above management and was discharged in a stable condition.
Discharge Medications: Prednisolone 20 mg, Hydroxychloroquine 200 mg, Leviteracetam 500 mg, Ramipril 5 mg, Carvedilol 3.125 mg
Final Diagnosis: Systemic Lupus Erythematosus, Lupus Nephritis, Probable CNS Vasculitis, Posterior Reversible Encephalopathy Syndrome (PRES), Myopericarditis
Discussion-
Systemic Lupus Erythematosus (SLE)
Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with a strong

Fig 1- Various etiologies of SLE
female predominance (10:1), typically affecting women aged 15–44 years, and is more prevalent among individuals of African descent. Its etiology is multifactorial, involving genetic predisposition (e.g., HLA-DR2, DR3; complement deficiencies), hormonal influences (e.g., estrogen exposure), and environmental triggers such as smoking, silica, UV light, and EBV. Certain drugs like procainamide and hydralazine can also induce lupus-like illness.
The pathogenesis involves defective clearance of apoptotic cells and immune complexes due to complement deficiencies, leading to autoantibody production (e.g., ANA, anti-dsDNA). SLE primarily manifests through type III hypersensitivity reactions, where immune complexes deposit in tissues, causing inflammation and organ damage. Type II hypersensitivity also contributes, particularly in SLE-associated cytopenias.
Systemic Lupus Erythematosus (SLE) is a chronic autoimmune disease marked by alternating phases of remission and relapse. It exhibits a wide spectrum of clinical presentations, ranging from mild constitutional symptoms such as fatigue, low-grade fever, and weight loss, to severe multi-organ involvement. The disease commonly affects

Fig 2- Clinical manifestations of SLE with its presenting frequencies
the joints (seen in over 90% of cases), presenting with arthritis, arthralgia, and distal symmetrical polyarthritis typically involving the fingers, wrists, and knees. Cutaneous manifestations are present in up to 85% of cases, including the classic malar (butterfly) rash, discoid rash, photosensitive maculopapular rashes, painless oral ulcers, non-scarring alopecia, Raynaud phenomenon, and periungual telangiectasias.
Less commonly, SLE can involve hematological systems (cytopenias with pallor, petechiae, or infections), muscles (myalgia), and serosal surfaces (pleuritis, pericarditis with effusions). Renal involvement may manifest as lupus nephritis with proteinuria. Cardiac complications include pericarditis, myocarditis, coronary artery disease, and Libman-Sacks endocarditis, which can affect the aortic or mitral valves. Pulmonary manifestations may include pneumonitis, interstitial lung disease, and pulmonary hypertension. Vascular involvement includes vasculitis and thromboembolic events, especially in the context of antiphospholipid syndrome. Neurological features range from seizures and psychosis to lupus cerebritis, aseptic meningitis, and peripheral neuropathies. Ocular symptoms like keratoconjunctivitis sicca may also occur.
Classification Criteria – SLICC

Fig 3- SLICC Criteria for SLE
Lupus Nephritis
Lupus nephritis is one of the most common and serious manifestations of SLE, affecting up to 60% of patients. The presence of proteinuria, hematuria, or rising creatinine should prompt further evaluation. The patient responded well to pulse steroids and cyclophosphamide, consistent with treatment for Class III-V nephritis.
Neuropsychiatric SLE – CNS Vasculitis and PRES in SLE
Neuropsychiatric manifestations occur in up to 40% of SLE patients. CNS vasculitis is rare but serious, and its diagnosis is supported by angiographic findings. PRES is a radiological diagnosis characterized by vasogenic edema, typically in parieto-occipital regions. In SLE, PRES is often triggered by hypertension, nephritis, and immunosuppression. Early recognition and control of blood pressure and underlying inflammation are essential.

Fig 4- ACR Classicifiaction of Neuropsychiatric syndromes in SLE
Myopericarditis in SLE
Cardiac involvement in SLE includes pericarditis, myocarditis, and endocarditis. Myopericarditis is suggested by elevated cardiac enzymes, global hypokinesia, and reduced EF. It is often underrecognized but may lead to heart failure if untreated.
Treatment-
In systemic lupus erythematosus (SLE), treatment is guided by clinical features and organ involvement. Hydroxychloroquine remains the cornerstone of therapy for all patients, regardless of disease activity. Oral glucocorticoids are commonly added to control symptoms, and immunosuppressive agents may be introduced in cases with more significant disease activity or inadequate response to initial therapy. In cases involving major organ manifestations such as nephritis, central nervous system involvement, or severe cytopenias, high-dose intravenous glucocorticoids are used in combination with potent immunosuppressants like cyclophosphamide, mycophenolate mofetil, or rituximab for induction of remission. Maintenance therapy typically includes hydroxychloroquine, with or without low-dose glucocorticoids and/or immunosuppressants or biologics. Disease flares are managed by intensifying therapy based on the severity and extent of organ involvement.
Importance of This Case
This case is significant due to the concurrent presentation of lupus nephritis, probable CNS vasculitis, PRES, myopericarditis, and urosepsis—a rare but life-threatening combination. It underscores the need for vigilance for multiorgan flares, early aggressive treatment, and multidisciplinary collaboration among rheumatology, nephrology, neurology, cardiology, and infectious disease specialists.

Fig 5- Treatement of SLE (Fierstein and Kelly’s textbook of Rheumatology)
Reference
- Fierstein and Kelly’s textbook of Rheumatology
- Harrison Principles of Internal Mediicne
- https://www.ncbi.nlm.nih.gov/books/NBK535405/ – Systemic Lupus Erythematosus Angel A. Justiz Vaillant; et al.
- https://www.ncbi.nlm.nih.gov/books/NBK499817/ – Lupus Nephritis Rina Musa; Preeti Rout; et al

Dr Vignesh A S
DNB GENERAL MEDICINE RESIDENT
Kauvery Hospital, Chennai

Dr Sham S (DM (Rheumatology)., MRCP (UK)., FRCP(Edin)., SCE (RCP UK)
Senior Consultant Rheumatology
Kauvery Hospital, Chennai