Systemic Lupus Erythematosus Features in Elderly Patients: Case-Based Review
Abstract
:1. Introduction
2. Case Presentation
3. Discussions
3.1. Epidemiology
3.2. Pathophysiology
3.2.1. Apoptosis and Toll-like Receptors (TLRs)
3.2.2. Innate Immune System
3.2.3. Adaptive Immune System
3.2.4. Complement System
3.2.5. Steroid Hormones
3.2.6. Physiopathology Particularities in Elderly Patients
3.3. Diagnosis
3.3.1. Diagnosis Criteria
3.3.2. Characteristics of SLE in the Elderly Patient
3.4. Management
3.5. Prognosis
4. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Conflicts of Interest
References
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Author, Year Number of Cases [Reference] | Age, Gender | Clinical Manifestation | Laboratory Test Abnormalities | Observations |
---|---|---|---|---|
So et al., 2019 3 cases [3] | 75 y/o ♂ | bilateral pleural effusion | positive tests for anti-dsDNA Ab hypocomplementemia pancytopenia, hematuria | In elderly patients with cardiovascular history, bilateral pleural effusion can mimic congestive heart failure. Thoracocentesis is essential for evaluating diuretic-resistant bilateral pleural effusion in elderly patients. |
80 y/o ♀ | bilateral pleural effusion | positive tests for ANA hypocomplementemia leukopenia, proteinuria | ||
83 y/o ♂ | bilateral pleural effusion | positive tests for ANA, anti-dsDNA Ab, elevated CRP levels | ||
Ikushima et al., 2018 1 case [4] | 85 y/o ♀ | fatigue, bilateral lower extremity edema, arthritis, pleural effusion, pericardial effusion | positive tests for ANA, anti-dsDNA Ab hypocomplementemia anemia, elevated ESR and CRP levels, elevated D-dimers levels | This report describes a case of very-late-onset SLE with serositis as the predominant clinical feature, highlighting the need to consider SLE in the differential diagnosis of rapidly accumulating pleural effusion in elderly patients. |
Hasegawa et al., 2023 1 case [7] | 87 y/o ♂ | anorexia, decreased food intake, bilateral pleural effusion, ascites, painful aphthous ulcers | positive tests for ANA hypocomplementemia leukopenia, thrombocytopenia, proteinuria | Diagnosing late-onset SLE in the elderly is challenging due to the prevalence of comorbidities and the need to distinguish it from other conditions. In some cases, conditions like pneumonia or heart failure may not fully explain the symptoms, leading to the eventual diagnosis of late-onset SLE through thorough testing and examinations. |
Fujita et al., 2018 1 case [9] | 70 y/o ♀ | gait disturbance, cognitive dysfunction, muscle rigidity, bilateral pleural effusion, bilateral lower extremity edema | positive tests for ANA, anti-dsDNA Ab, anti-La/SS-B Ab hypocomplementemia lymphocytopenia, anemia, thrombocytopenia, elevated ESR and ferritin levels | Differentiating causes of cognitive impairment in elderly patients is challenging, with neurodegenerative diseases like Alzheimer’s being common considerations. Neuropsychiatric SLE should also be included in the differential diagnosis. Prompt and appropriate immunosuppressive therapy can potentially reverse cognitive dysfunction. |
Kim et al., 2024 1 case [13] | 81 y/o ♀ | photosensitive lesions in sun-exposed sites | positive tests for ANA, anti-Ro/SS-A Ab | This case illustrates the development of SCLE after starting osimertinib for non-small-cell lung cancer. Prompt recognition and management of cutaneous side effects are crucial to maintaining targeted cancer therapy. |
Mrabet et al., 2024 1 case [14] | 82 y/o ♂ | fatigue, dyspnea, abdominal pain, hepatosplenomegaly, pericardial effusion | positive tests for ANA, anti-nucleosome Ab, anti-histone Ab hypocomplementemia anemia, acute kidney injury, proteinuria, hematuria, hepatic cholestasis | Late-onset SLE often presents insidiously with atypical features, leading to delayed diagnosis, as seen in this case, who had abdominal pain and dyspnea without typical skin or joint signs. Moreover, this case demonstrates that familial late-onset SLE can occur in elderly men, presenting with a relatively mild form that does not necessitate intensive immunosuppressive therapy. |
Tsuji et al., 2024 1 case [15] | 75 y/o ♂ | pleural effusion, pericardial effusion, gait ataxia, hand dexterity impairments, arthralgias | positive tests for ANA, anti-Ro/SS-A Ab, RF lymphocytopenia, anemia, elevated CRP levels | This case emphasizes that late-onset SLE can present with diverse symptoms, including unilateral pleural effusion and neurological issues, and should be considered when the cause of pleural effusion is unclear. |
Rodriguez- Perez, 2024 1 case [16] | 90 y/o ♀ | recurrent unilateral pleural effusion, pericardial effusion, arthralgias, bilateral lower extremity edema | positive tests for ANA, p-ANCA anemia, marked proteinuria | This case emphasizes the significance of recognizing autoimmune conditions such as SLE in elderly patients over 90 y/o, with the presentation of marked proteinuria representing a rare and atypical manifestation. |
Kioi et al., 2023 1 case [17] | 86 y/o ♂ | persistent unilateral pleural effusion | positive tests for ANA, anti-dsDNA Ab, anti-cardiolipin immunoglobulin G Ab, anti-U1-RNP Ab, anti-La/SS-B Ab, anti-CCP Ab lymphocytopenia, elevated CRP levels | The growing number of long COVID cases raises concerns about undiagnosed autoimmune conditions, such as lupus. In patients with persistent pleural effusion or lymphocytopenia post-COVID-19, testing for SLE-specific autoantibodies, including anti-dsDNA and anti-Sm Ab, should be considered. |
Chao et al., 2022 1 case [18] | 82 y/o ♀ | ascites, pleural effusion, spontaneous oral bleeding, multiple ecchymoses | positive tests for ANA, anti-Sm Ab hypocomplementemia normocytic anemia, thrombocytopenia, renal dysfunction, proteinuria, elevated CA-125 levels | Pseudo-pseudo Meigs’ syndrome should be considered as a key differential diagnosis in female patients presenting with ascites, pleural effusion, and elevated CA-125 levels. The initial evaluation should focus on ruling out pelvic tumors associated with Meigs’ or pseudo-Meigs’ syndrome. An SLE flare should be considered, even in elderly patients. |
Helali et al., 2022 1 case [19] | 75 y/o ♂ | weakness, dyspnea, respiratory failure | positive tests for ANA, anti-dsDNA Ab anemia, thrombocytopenia, proteinuria | Non-infectious fulminant lupus pneumonitis is a rare, severe syndrome with high mortality, with only a few cases reported in the literature, particularly in young patients. It is often challenging to diagnose, especially as an initial SLE manifestation. |
Xu et al., 2021 1 case [20] | 77 y/o ♂ | unilateral pleural effusion, shortness of breath over the past 3 years | positive tests for ANA, anti-dsDNA Ab, MPO-ANCA anemia, renal dysfunction, proteinuria, microscopic hematuria | The overlap syndrome of SLE and antineutrophil cytoplasmic antibody-associated vasculitis is a rare condition. It is marked by the presence of serological markers and severe clinical features, such as rapidly progressive glomerulonephritis and pulmonary involvement, meeting the diagnostic criteria for both diseases. This syndrome predominantly affects women of childbearing age. |
Kuroda et al., 2021 1 case [21] | 78 y/o ♀ | fatigue, diplopia, left sixth cranial nerve palsy, polyarthritis | positive tests for ANA, anti-dsDNA Ab, lupus anticoagulant hypocomplementemia leukopenia, anemia, elevated erythrocyte sedimentation rate (ESR) levels | This case highlights a challenging diagnostic scenario involving an elderly woman with autoimmune hemolytic anemia and isolated sixth cranial nerve palsy. The advanced age, low ANA titers, and atypical presentation made the diagnosis of SLE particularly complex. |
Constantinescu et al., 2021 2 cases [22] | 81 y/o ♀ | vesperal fever, weight loss, arthralgias, myalgias, unilateral pleural effusion, pericardial fluid, atrophic erythematous squamous plaques | positive tests for ANA, anti-dsDNA Ab, anti-Ro/SS-A Ab leukopenia with lymphocytopenia, normocytic anemia | Skin lesions are less commonly reported in late-onset lupus, with malar rash being particularly rare in this group. However, the two cases discussed showed chronic and subacute cutaneous lupus lesions, later confirmed by skin biopsy. |
72 y/o ♀ | weight loss, recurring fever, arthralgias, rounded erythematous squamous plaques | positive tests for ANA, anti-dsDNA Ab, RF leukopenia with lymphocytopenia, anemia | ||
Bao et al., 2021 1 case [23] | 84 y/o ♂ | bilateral peripheral edema | positive tests for ANA, anti-dsDNA Ab hypocomplementemia renal dysfunction, hypoalbuminemia, proteinuria, microscopic hematuria, pyuria | This case highlights a rare presentation of lupus nephritis as a paraneoplastic manifestation of mantle cell lymphoma. The rapid remission of renal and immune symptoms following chemotherapy supports the secondary nature of the lupus nephritis. |
Tay et al., 2020 1 case [24] | 71 y/o ♀ | fever, alopecia, hepatomegaly, multiple intra-abdominal and intra-thoracic lymphadenopathies | positive tests for ANA, anti-dsDNA Ab leukopenia, anemia, renal dysfunction, microscopic hematuria, proteinuria | This case highlights the established connection between rheumatological disorders and cancers, as evidenced by unusual clinical features such as pronounced hepatomegaly and extensive lymphadenopathy, suggesting the presence of an underlying malignancy. Angioimmunoblastic T-cell lymphoma is a rare subtype of non-Hodgkin lymphoma. Its diagnosis is challenging due to subtle histological features. |
Rezazadegan et al., 2020 1 case [25] | 78 y/o ♀ | papulosquamous itching skin lesions, weight loss, heartburn, arthralgias, myalgias | positive tests for ANA, anti-Ro/SS-A Ab hypocomplementemia anemia, biopsy of gastric mass showing gastric adenocarcinoma | Rheumatic diseases, like dermato-myositis, have been well established as paraneoplastic syndromes. More recently, growing evidence has linked SCLE to various malignancies, primarily solid tumors. These cancers may arise before, simultaneously with, or after the onset of SCLE. |
Bonemetti et al., 2020 1 case [26] | 85 y/o ♀ | hemodynamic instability, diffuse dyscrasia edemas, peripheral cyanosis, particularly on her fingers, pleural effusion | positive tests for ANA, anti-Ku Ab, ANCA leukocytosis with lymphocytopenia, thrombocytopenia, renal dysfunction, elevated CRP and ferritin levels | This is the first documented case in the literature of SLE with vasculitis triggered by COVID-19 infection. |
de Montjoye et al., 2020 1 case [27] | 81 y/o ♀ | impaired spatial and time orientation, dyspnea, weight loss, fever, arthritis, facial rash | positive tests for ANA, anti-dsDNA Ab, anti-Ro/SS-A Ab anemia, hypoalbuminemia, proteinuria, elevated CRP levels | Late-onset SLE is an uncommon but significant cause of reversible functional and cognitive decline in elderly patients. Its presentation in this age group is often atypical, making the diagnosis challenging. |
Kalenchic et al., 2019 1 case [28] | 75 y/o ♀ | dyspnea, low-grade fever, arthritis, pericardial effusion, pleural effusion | positive tests for ANA, anti-dsDNA Ab, anti-nucleosome Ab lymphocytopenia, anemia, elevated ESR and CRP levels | This case illustrates that late-onset SLE can be a potential cause of pleural fluid accumulation in elderly patients. |
D’Andréa et al., 2018 1 case [29] | 82 y/o ♀ | anorexia, weight loss, fatigue, arthralgia, unilateral pleural effusion | positive tests for ANA, anti-nucleosome Ab leukopenia with eosinophilia, anemia, elevated CRP levels, pleural effusion cytological examination showing Hargraves cells | In elderly patients, eosinophilic pleural effusion is most often linked to malignancies and infections. In rare instances, pleural eosinophilia may be associated with connective tissue diseases. When accompanied by chronic joint pain and hematologic abnormalities, SLE should be considered in the differential diagnosis. |
Jatwani et al., 2018 1 case [30] | 81 y/o ♂ | shortness of breath, pericardial effusion, bilateral pleural effusion, bilateral lower extremity edema | positive tests for ANA, anti-dsDNA Ab, anti-beta-2 glycoprotein IgA, anti-CCP hypocomplementemia anemia, thrombocytopenia | Bronchiolitis obliterans organizing pneumonia (BOOP) is a rare manifestation of connective tissue disorders like SLE. In this case, BOOP was diagnosed 8 years before SLE. The high relapse rate, despite adequate steroid therapy, suggests a causal link to underlying SLE and indicates BOOP as the initial presentation. |
Arai et al., 2018 1 case [31] | 74 y/o ♂ | fever, dyspnea, pericardial effusion, pleural effusion, arthritis | positive tests for ANA, anti-dsDNA Ab hypergammaglobulinemia, hypocomplementemia renal dysfunction | This report highlights a unique case of comorbid autoimmune diseases, featuring the coexistence of IgG4-related disease (IgG4-RD) and SLE. The clinical course demonstrated a transition from ANA-positive IgG4-RD to SLE after the surgical removal of gastric cancer. |
Shirai et al., 2017 2 cases [32] | 82 y/o ♂ | bilateral polyarthritis | positive tests for ANA, anti-dsDNA Ab lymphocytopenia, mild thrombocytopenia, renal dysfunction, proteinuria | These two cases demonstrated that very-late-onset male SLE can present with atypical features, such as the absence of characteristic skin lesions, severe cytopenia, hypocomplementemia, anti-SS-A, anti-RNP, or anti-Sm antibodies, which complicate the diagnosis of SLE. |
83 y/o ♂ | fatigue, dyspnea, bilateral pleural effusion, bilateral lower extremity edema | positive tests for ANA, anti-dsDNA Ab lymphocytopenia, hypoalbuminemia, elevated ESR and CRP levels | ||
Boddu et al., 2016 1 case [33] | 73 y/o ♀ | painful blistering skin rash, oral ulcers | positive tests for ANA, anti-RNP Ab, anti-Sm Ab hypocomplementemia anemia, thrombocytopenia, hypoalbuminemia, proteinuria | This case highlights the challenges physicians face in diagnosing among the numerous immunobullous dermatoses. Bullous SLE must be especially distinguished from pemphigoid and epidermolysis bullosa acquisita. |
Kamiya et al., 2016 1 case [34] | 83 y/o ♀ | bilateral pleural effusion | positive tests for ANA, anti-dsDNA Ab, anti-RNP Ab hypocomplementemia proteinuria, elevated CRP levels, elevated CA-125 levels | Some studies have suggested a potential link between anti-RNP antibodies and lupus serositis. These findings indicate that lupus serositis should be considered in patients with anti-RNP-positive interstitial pneumonia. |
Hammami et al., 2014 1 case [35] | 77 y/o ♀ | massive ascites, unilateral exudative pleuritis 4 years prior to presentation | positive tests for ANA, anti-dsDNA Ab, anti-Sm Ab hypocomplementemia leukopenia with lymphocytopenia, anemia, hypoalbuminemia | Massive ascites is a rare presentation of SLE. Lupus peritonitis may mimic acute abdominal conditions or present chronically as persistent painless ascites, chronic pancreatitis, or mild abdominal pain. |
Agguire et al., 2014 1 case [36] | 72 y/o ♂ | right-sided chest pain, dyspnea, erythematous lesions on the sun-exposed areas, oral ulcers, bilateral lower and upper extremity edema, massive unilateral pleural effusion | positive tests for ANA, anti-dsDNA Ab, anti-RNP Ab hypocomplementemia leukopenia with lymphocytopenia, elevated CRP levels | Diagnosing late-onset SLE in elderly patients is challenging due to its nonspecific presentation and broad differential diagnoses. While elderly-onset SLE typically shows lower rates of anti-dsDNA, anti-RNP Ab, and hypocomplementemia, this case demonstrated elevated levels of these markers alongside complement consumption. |
Chebbi et al., 2014 1 case [37] | 82 y/o ♀ | anorexia, weight loss, fatigue, arthralgia, unilateral pleural effusion | positive tests for ANA, anti-nucleosome Ab leukopenia with eosinophilia, anemia, elevated CRP levels, pleural effusion cytological examination showing Hargraves cells | In elderly patients, eosinophilic pleural effusion is most often linked to malignancies and infections. In rare instances, pleural eosinophilia may be associated with connective tissue diseases. When accompanied by chronic joint pain and hematologic abnormalities, SLE should be considered in the differential diagnosis. |
Iyoda et al., 2008 1 case [38] | 86 y/o ♂ | general malaise | positive tests for ANA, lupus anticoagulant anemia, thrombocytopenia, elevated activated partial thromboplastin time, hypofibrinogenemia, elevated fibrin degradation products, elevated D-dimers, renal dysfunction, proteinuria, elevated CRP levels | The presence of disseminated intravascular coagulation (DIC) without an identifiable cause should raise suspicion for SLE, even in elderly patients. There have been only a few reports of DIC as the initial manifestation of SLE. |
Ito et al., 2002 1 case [39] | 77 y/o ♀ | massive ascites, bilateral pleural effusion, pericardial effusion, bilateral lower extremity edema | positive tests for ANA, anti-dsDNA Ab hypocomplementemia leukopenia, anemia, renal dysfunction, hypoalbuminemia, proteinuria, microscopic hematuria | Chronic lupus peritonitis in elderly patients may show limited response to glucocorticoid therapy due to persistent peritoneal inflammation and impaired vascular function. |
Aharon et al., 1994 2 cases [40] | 70 y/o ♂ | pleural effusion, generalized maculopapular rash 3 years prior to presentation | positive tests for ANA, anti-dsDNA Ab, anti-Sm Ab hypocomplementemia pancytopenia, renal dysfunction, proteinuria | SLE should be considered in elderly patients presenting with multiorgan autoimmune disease. For elderly patients where other immunosuppressive therapies are contraindicated, low-dose monthly intravenous immunoglobulin may be a treatment option. |
82 y/o ♀ | anorexia, weakness, nodular purplish generalized skin rash, pericardial effusion, recurrent pleural effusion and recurrent chondritis over a period of 9 years prior to presentation | hypocomplementemia pancytopenia, renal dysfunction, elevated ESR levels |
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Ceasovschih, A.; Alexa, R.-E.; Șorodoc, V.; Asaftei, A.; Stoian, D.C.; Morărașu, B.C.; Balta, A.; Lionte, C.; Stoica, A.; Sîrbu, O.; et al. Systemic Lupus Erythematosus Features in Elderly Patients: Case-Based Review. J. Clin. Med. 2025, 14, 2558. https://doi.org/10.3390/jcm14082558
Ceasovschih A, Alexa R-E, Șorodoc V, Asaftei A, Stoian DC, Morărașu BC, Balta A, Lionte C, Stoica A, Sîrbu O, et al. Systemic Lupus Erythematosus Features in Elderly Patients: Case-Based Review. Journal of Clinical Medicine. 2025; 14(8):2558. https://doi.org/10.3390/jcm14082558
Chicago/Turabian StyleCeasovschih, Alexandr, Raluca-Elena Alexa, Victorița Șorodoc, Andreea Asaftei, Denisa Cristiana Stoian, Bianca Codrina Morărașu, Anastasia Balta, Cătălina Lionte, Alexandra Stoica, Oana Sîrbu, and et al. 2025. "Systemic Lupus Erythematosus Features in Elderly Patients: Case-Based Review" Journal of Clinical Medicine 14, no. 8: 2558. https://doi.org/10.3390/jcm14082558
APA StyleCeasovschih, A., Alexa, R.-E., Șorodoc, V., Asaftei, A., Stoian, D. C., Morărașu, B. C., Balta, A., Lionte, C., Stoica, A., Sîrbu, O., Constantin, M., Diaconu, A.-D., Lăcătușu, C.-M., Grigorescu, E.-D., & Șorodoc, L. (2025). Systemic Lupus Erythematosus Features in Elderly Patients: Case-Based Review. Journal of Clinical Medicine, 14(8), 2558. https://doi.org/10.3390/jcm14082558