Draft:Signs and symptoms of lupus
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The presentation of lupus varies from person to person.[2] Lupus is considered “the most clinically and serologically diverse autoimmune disease” since it causes a wide range of symptoms and can affect almost any organ.[3] The symptoms of lupus can range from mild to severe.[3] Lupus usually has a pattern of high activity, known as flares, and periods with milder symptoms. The duration and intensity of these flares vary amongst individuals and cannot be predicted.[2] The most common signs and symptoms of lupus are constitutional symptoms such as fatigue, malaise or widespread pain, fever, anorexia, and lymphadenopathy, however, these symptoms are non-specific and don’t help distinguish lupus from other disorders.[4] Any part of the body may be affected by lupus however it mainly affects the skin, kidneys, joints, nervous system, lungs, muscles, and blood.[5]
Constitutional symptoms
[edit]Constitutional symptoms are extremely common with lupus; however, due to their nonspecific nature, they aren’t part of the classification criteria. The most common constitutional symptom with lupus is fatigue; other constitutional symptoms, including fever, lymphadenopathy, splenomegaly, and anorexia, may also occur.[6]
Fatigue affects up to 80% to 90% of those with lupus and is one of the most common and debilitating symptoms.[5] Fatigue in lupus is caused by a number of factors such as physical inactivity, sleeping patterns, psychological factors, weight, disease activity, and comorbid disorders.[8][6] Fatigue often continues after treatment of an acute flare and typically gets worse as the day goes on.[5]
Along with fatigue, many people with lupus have brain fog or cognitive dysfunction. Also termed “lupus fog”, brain fog can present as issues with concentration, memory, or difficulties thinking clearly. The prevalence of brain fog in lupus is between 3% to 88%.[8]
Although fever is a common symptom of lupus,[8] affecting between 36% and 86% of those with lupus,[5] the prevalence of fever in those with lupus has decreased over the years.[9] This may be due to the increased use and availability of nonsteroidal anti-inflammatory drugs and steroids[8] or physicians' increased awareness of excluding other potential causes of fevers, such as infections or malignancies.[9] Fever is more common in the early stages of lupus[8] and childhood-onset lupus than in late-onset lupus.[9]
The prevalence of lymphadenopathy is thought to be between 5% and 7% at the onset of lupus and between 12% and 15% at any point during the course of lupus. Lymphadenopathy is more prevalent during the initial years of lupus and less frequent as the disease progresses.[10] The lymph nodes in lupus lymphadenopathy are soft, movable, painful, and nonadherent to deep planes. Lupus primarily affects the cervical and axillary regions.[8]
Splenomegaly affects 10–45% of those with lupus and is more prevalent when the disease is active. Gross or big splenic enlargement is relatively uncommon and splenomegaly typically occurs in mild to moderate degrees.[11]
Loss of appetite and unexplained weight loss are both symptoms of lupus.[5][12] Weight loss can be due to lupus itself, gastrointestinal symptoms such as nausea and vomiting, or complications of lupus.[11][5] Weight loss affects 17% to 51% of those with lupus.[12]
Musculoskeletal symptoms
[edit]The most commonly affected organ system in lupus is the musculoskeletal system.[4] Around 90% of those with lupus experience arthritis.[13] There are three common patterns of arthritis in lupus, nonerosive arthritis, erosive arthritis (also called “rhupus”), and Jaccoud’s arthropathy.[14] Alongside arthritis, those with lupus commonly experience arthralgias and widespread muscular pain.[15] With lupus, muscle involvement can range from less common inflammatory myopathy or myositis to more prevalent myalgia.[16]
Lupus arthritis usually affects the smaller joints in the hands,[18] wrists and knees, and is symmetric.[19] Symptoms of lupus arthritis include pain, erythema, swelling, warmth, tenderness, and morning stiffness.[14] Lupus arthritis is often non-erosive and nondeforming.[19][4]
A small portion of those with lupus end up developing erosive arthritis with synovitis, called “rhupus”.[18][20] Those with rhupus fufill criteria for a diagnosis of rheumatoid arthritis and lupus.[21][22]
Jaccoud arthropathy is a type of non-erosive and deforming arthritis that can form in some people with lupus.[23] It usually affects the hands but can affect other joints such as the feet and knees.[24] The most common joint deformities caused by Jaccoud arthropathy are swan neck, ulnar deviation, thumb subluxation (z-thumb), hallux valgus, and boutonniere deformity.[25]
Muscle involvement in lupus can range from muscle pain and tenderness to myositis.[26] Muscle pain affects over 50% of those with lupus while inflammatory myositis affects less than 10% of those with lupus.[20][27] Lupus myositis presents similarly to idiopathic inflammatory myopathy and can cause muscle weakness, muscle pain, and elevated muscle enzymes.[28] Rarely, lupus can cause orbital myositis which can cause unilateral or bilateral diplopia or pain.[29]
Avascular necrosis (AVN), also known as osteonecrosis, ischemic necrosis of bone, osteochondriits dissecans, and aseptic necrosis is the death of trabecular bone and bone marrow which can damage the structure of bones.[30] AVN can cause pain and disability[31] or be asymptomatic. AVN affects up to 30% of those with lupus[27] and usually affects the epiphysis of longer bones such as the bones in the hips and knees.[29]
Lupus most commonly affects the tendons by causing tenosynovitis, however, it can also cause tendon dislocation, tendon tear, tendonitis, enthesitis, and tendon thinning.[16] Those with lupus may have a higher risk of localized soft tissue disorders due to deconditioning, fatigue, and weakness. Those with lupus often experience lax connective tissue. 5% to 12% of those with lupus have subcutaneous nodules. Those with lupus are more prone to infections including infections in the musculoskeletal system such as osteomyelitis, septic arthritis, pyomyositis, and soft tissue infections such as necrotizing fasciitis.[29] Osteoporosis is common in lupus and the prevalence ranges from 4.0% to 48.8%. Osteoporosis can lead to a higher risk of fractures.[32]
Mucocutaneous symptoms
[edit]Cutaneous manifestations of lupus are the second most common symptom and occur in 70% to 80% of those with lupus.[33] Those with lupus may experience a wide range of skin symptoms that can vary in severity and duration.[18] In lupus, skin lesions are split into two groups: lupus-specific and lupus-nonspecific manifestations.[34][33]
Lupus-specific skin lesions
[edit]Lupus-specific skin lesions include acute cutaneous lupus erythematosus (ACLE), subacute cutaneous lupus erythematosus (SCLE), and chronic cutaneous lupus erythematosus (CCLE).[27]
Lupus-nonspecific skin lesions
[edit]Lupus-nonspecific skin lesions can occur with other disorders and are not limited to lupus. Lupus-nonspecific skin lesions are more commonly seen in lupus compared to lupus-specific symptoms and include vasculitis, vascular lesions, livedo reticularis, papular mucinosis, alopecia, and nail changes.[35][34][33]
References
[edit]- ^ Shiel 2023.
- ^ a b Kyttaris 2024, p. 156.
- ^ a b Kehl & Wallace 2025, p. 413.
- ^ a b c Aranow, Diamond & Mackay 2023, p. 664.
- ^ a b c d e f Rudinskaya, Reyes-Thomas & Lahita 2021, p. 305.
- ^ a b Shaharir & Gordon 2021, p. 351.
- ^ Dey, Parodis & Nikiphorou 2021, p. 3.
- ^ a b c d e f Kehl & Wallace 2025, p. 417.
- ^ a b c Shaharir & Gordon 2021, p. 353.
- ^ Shaharir & Gordon 2021, p. 355.
- ^ a b Shaharir & Gordon 2021, p. 356.
- ^ a b Kehl & Wallace 2025, p. 418.
- ^ Sandhu, Torralba & Cabling 2025, p. 433.
- ^ a b Barilla-LaBarca et al. 2021a, p. 541.
- ^ Kyttaris 2024, p. 159.
- ^ a b Barilla-LaBarca et al. 2021b, p. 363.
- ^ Unsal, Arli & Akman 2007, p. 3.
- ^ a b c Kyttaris 2024, p. 157.
- ^ a b Barilla-LaBarca et al. 2021b, p. 361.
- ^ a b Rudinskaya, Reyes-Thomas & Lahita 2021, p. 306.
- ^ Antonini et al. 2020, p. 2.
- ^ Sandhu, Torralba & Cabling 2025, p. 434.
- ^ Kyttaris 2024, pp. 157–158.
- ^ Barilla-LaBarca et al. 2021a, p. 543.
- ^ Barilla-LaBarca et al. 2021b, p. 362.
- ^ Barilla-LaBarca et al. 2021a, p. 544.
- ^ a b c Aranow, Diamond & Mackay 2023, p. 665.
- ^ Barilla-LaBarca et al. 2021b, p. 364.
- ^ a b c Sandhu, Torralba & Cabling 2025, p. 436.
- ^ Barilla-LaBarca et al. 2021a, p. 546.
- ^ Barilla-LaBarca et al. 2021b, p. 365.
- ^ Sandhu, Torralba & Cabling 2025, p. 437.
- ^ a b c Concha & Werth 2021, p. 447.
- ^ a b Kuhn, Landmann & Bonsmann 2021, p. 372.
- ^ Chong & Werth 2025, p. 426.
Works cited
[edit]- Shiel, William C. Jr. (2023-11-08). "Lupus Causes, Symptoms, Treatment, Medications, Prevention". MedicineNet. Retrieved 2024-11-11.
- Kyttaris, Vasileios C. (2024). "Systemic lupus erythematosus". The Rose and Mackay Textbook of Autoimmune Diseases. Elsevier. pp. 149–172. doi:10.1016/b978-0-443-23947-2.00016-3. ISBN 978-0-443-23947-2.
- Kehl, Amy; Wallace, Daniel J. (2025). "Overview and clinical presentation". Dubois' Lupus Erythematosus and Related Syndromes. Elsevier. doi:10.1016/b978-0-323-93232-5.00039-3. ISBN 978-0-323-93232-5.
- Aranow, Cynthia; Diamond, Betty; Mackay, Meggan (2023). "Systemic Lupus Erythematosus". Clinical Immunology. Elsevier. doi:10.1016/b978-0-7020-8165-1.00052-6. ISBN 978-0-7020-8165-1.
- Rudinskaya, Alla; Reyes-Thomas, Joyce; Lahita, Robert G. (2021). "The clinical presentation of systemic lupus erythematosus and laboratory diagnosis". Lahita's Systemic Lupus Erythematosus. Elsevier. doi:10.1016/b978-0-12-820583-9.00002-6. ISBN 978-0-12-820583-9.
- Shaharir, Syahrul Sazliyana; Gordon, Caroline (2021). "Constitutional symptoms and fatigue in systemic lupus erythematosus". Systemic Lupus Erythematosus. Elsevier. doi:10.1016/b978-0-12-814551-7.00039-8. ISBN 978-0-12-814551-7.
- Dey, Mrinalini; Parodis, Ioannis; Nikiphorou, Elena (2021-08-13). "Fatigue in Systemic Lupus Erythematosus and Rheumatoid Arthritis: A Comparison of Mechanisms, Measures and Management". Journal of Clinical Medicine. 10 (16). MDPI: 3566. doi:10.3390/jcm10163566. ISSN 2077-0383. PMC 8396818. PMID 34441861.
- Sandhu, Vaneet; Torralba, Karina; Cabling, Marven (2025). "The musculoskeletal system and bone metabolism". Dubois' Lupus Erythematosus and Related Syndromes. Elsevier. doi:10.1016/B978-0-323-93232-5.00041-1. ISBN 978-0-323-93232-5.
- Barilla-LaBarca, Maria-Louise; Horowitz, Diane; Marder, Galina; Furie, Richard (2021). "Musculoskeletal system: articular disease, myositis, and bone metabolism". Lahita's Systemic Lupus Erythematosus. Elsevier. doi:10.1016/b978-0-12-820583-9.00022-1. ISBN 978-0-12-820583-9.
- Barilla-LaBarca, Maria-Louise; Horowitz, Diane; Marder, Galina; Furie, Richard (2021). "The musculoskeletal system in SLE". Systemic Lupus Erythematosus. Elsevier. doi:10.1016/b978-0-12-814551-7.00040-4. ISBN 978-0-12-814551-7.
- Unsal, Erbil; Arli, Ayse Ozgun; Akman, Hakki (2007-05-04). "Rhupus arthropathy as the presenting manifestation in Juvenile SLE: a case report". Pediatric Rheumatology Online Journal. 5. Springer: 7. doi:10.1186/1546-0096-5-7. ISSN 1546-0096. PMC 1887527. PMID 17550633.
- Antonini, Luca; Le Mauff, Brigitte; Marcelli, Christian; Aouba, Achille; de Boysson, Hubert (September 2020). "Rhupus: a systematic literature review". Autoimmunity Reviews. 19 (9). Elsevier: 102612. doi:10.1016/j.autrev.2020.102612. PMID 32668290.
- Concha, Josef Symon S.; Werth, Victoria P. (2021). "Skin". Lahita's Systemic Lupus Erythematosus. Elsevier. doi:10.1016/b978-0-12-820583-9.00008-7. ISBN 978-0-12-820583-9.
- Kuhn, Annegret; Landmann, Aysche; Bonsmann, Gisela (2021). "Cutaneous lupus erythematosus". Systemic Lupus Erythematosus. Elsevier. doi:10.1016/b978-0-12-814551-7.00041-6. ISBN 978-0-12-814551-7.
- Chong, Benjamin F.; Werth, Victoria P. (2025). "Skin disease in cutaneous lupus erythematosus". Dubois' Lupus Erythematosus and Related Syndromes. Elsevier. doi:10.1016/b978-0-323-93232-5.00040-x. ISBN 978-0-323-93232-5.