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Symptoms and Complications of Lupus

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Overview
Antiphospholipid
Atherosclerosis
Brain, Cognitive
Cachexia
Calcinosis
Cancer
Cytomegalovirus
Dermatofibromas
Fatigue
Fibromyalgia and Lupus
Flares
Gastrointestinal
Hearing
Heart Disease
Jaccoud's Arthritis
Kidney
Liver
Lymphoma
Microstomia
Miscarriages
Musculoskeletal
Neuropsychiatric
Pancreatitis
Persistent Lupus Myelitis
Pulmonary
Retinal
Skin
Sleep Disorders
Thrombocytopenia
Thyroid
Tuberculosis
Vascular

Overview

Photo of butterfly plaqueSystemic lupus erythematosus (SLE) can affect any part of the body, including blood vessels, and internal organs.

It can also cause cognitive impairments, neuropathies, psychosis, and depression.

It is perhaps most widely known for the distinctive butterfly (malar) facial rash that can occur during flares or after sun exposure. Oddly enough, however, the butterfly rash only occurs in about 50% of lupus patients. Therefore, the absence of malar rash does not preclude a lupus diagnosis.

General symptoms, that occur with many other diseases as well, include muscle and joint pain, Raynaud's, fatigue, low-grade fever, mouth and nose ulcers, and loss of appetite. (Also see Autoimmune Diseases and Overview of Lupus)

What are the symptoms of lupus? MedicineNet

A retrospective study of joint infections in patients with systemic lupus erythematosus (SLE). When an SLE patient presents with pre–existing arthritis and suddenly develops asymmetric oligo– or large–joint swelling and pain with elevated C-reactive protein levels and low disease activity, joint infections should be considered. PubMed, Clin Rheumatol, 07/08/2017.

Antiphospholipid Syndrome (APS)

Antiphospholipid Syndrome is also known as Hughes Syndrome. ISN.

Overview
APS and Heart Disease
Scleroderma and APS
Diagnosis
Symptoms
Complications
Treatments
Causes and Research
Support Resources
Personal Stories

Atherosclerosis

Biomarkers associating endothelial Dysregulation in pediatric-onset systemic lupus erythematous (pSLE). Endothelial dysregulation associating proteins seems to be potent biomarkers for pSLE activity as well as organ involvement in pSLE patients. PubMed, Pediatr Rheumatol Online J, 2019 Oct 24;17(1):69.

Incidence and predictors of aorta calcification in patients with systemic lupus erythematosus. Systemic lupus erythematosus patients had a much earlier onset and higher incidences of aorta calcium than coronary artery calcification. PubMed, Lupus, 2019 Feb 10:961203319826689.

Cardiovascular Disease in Lupus. People with lupus have a significantly increased risk of premature coronary heart disease (CHD) or atherosclerosis, stroke and other cardiovascular-related conditions than those without lupus. LupusResearchUpdate.

Brain, Cognitive

Blood–brain barrier (BBB) leakage in systemic lupus erythematosus (SLE) is associated with gray matter loss and cognitive impairment. Our findings provide evidence for a link between extensive BBB leakage and changes in both brain structure and cognitive function in patients with SLE. PubMed, Ann Rheum Dis, 10/01/2020.

Cerebrovascular Events (CerVEs) in Systemic Lupus Erythematosus (SLE). CerVEs, the fourth most frequent neuropsychiatric event in SLE, are usually attributable to lupus. PubMed, Arthritis Care Res (Hoboken), 01/05/2018.

Enlarged perivascular spaces (EPVS) are associated with the disease activity in systemic lupus erythematosus. The centrum semiovale (CS)–EPVS in the SLE patients are associated with the systemic disease activity, suggesting that CS–EPVS may be indicative of the reactive changes of the white matter due to the inflammatory activity. ResearchGate, December 2017.

Impact of cognitive impairment, depression, disease activity, and disease damage on quality of life (HRQoL) in women with systemic lupus erythematosus (SLE). Our results highlight the relevance of executive dysfunction to poor physical and mental health components of HRQoL in SLE together with depression, while disease activity and disease damage are associated with lower HRQoL physical components. PubMed, Scand J Rheumatol, 2017 Jul;46(4):273-280.

Clinical utility of circulating anti-N-methyl-d-aspartate receptor subunits NR2A/B antibody for the diagnosis of neuropsychiatric (NP) syndromes in systemic lupus erythematosus (SLE) and Sjögren's syndrome (SS). Circulating anti-NR2A/B antibody testing has a diagnostic value for NP syndromes in SLE and SS collectively. PubMed, Autoimmun Rev, 2017 Feb;16(2):114-122. (Also see Sjogren's Syndrome)

Cachexia

Cachexia in Systemic Lupus Erythematosus (SLE): Risk Factors and Relation to Disease Activity and Damage. Cachexia is an underrecognized syndrome in patients with SLE and SLE patients with intermittent cachexia have the highest risk of future organ damage. PubMed, Arthritis Care Res (Hoboken), 08/02/2020.

Calcinosis

(Case Report) Diffuse Dystrophic Calcinosis Cutis of the Scalp in a Patient With Scalp Discoid Lupus Erythematosus and Systemic Lupus Erythematosus. PubMed, JAMA Dermatol.

Cancer

Cervical Cancer Screening in Women with Systemic Lupus Erythematosus (SLE). Patients with SLE are at higher risk of abnormal cervical screening test results than controls, supporting the need for regular screening. PubMed, Arthritis Care Res (Hoboken), 08/16/2020. (Also see Cancer)

Cytomegalovirus

Disseminated cytomegalovirus (CMV) infection complicating active treatment of systemic lupus erythematosus: an emerging problem. Given the rise in prevalence of CMV, there is a need for appropriate screening for this opportunistic pathogen and studies to determine the risks and benefits of prophylactic or preemptive treatment for this virus. PubMed, Lupus, 2017 Apr;26(4):431-434.

Dermatofibromas in Lupus

Case Report: Multiple dermatofibromas in a patient with systemic lupus erythematosus (SLE) and Sjögren's syndrome (SS). As these tumors appeared during the remission stage of SLE, they might have been under immunosuppressive conditions caused by immunosuppressing agents rather than collagen disease itself. SpringerLink. (Also see Sjogren's)

Fatigue in Lupus

Kynurenine pathway is altered in patients with systemic lupus erythematosus (SLE) and associated with severe fatigue. Metabolites in the kynurenine pathway are altered in patients with SLE compared with controls. PubMed, Lupus Sci Med, 2018 May 23;5(1):e000254.

Fibromyalgia and Lupus

What's the difference between lupus and fibromyalgia? Fibromyalgia can occur concurrently with lupus, but most of the time lupus and fibromyalgia are separate diagnoses. Lupus Foundation of America.

Flares of Lupus

Analysis of Erythrocyte C4d to Complement Receptor 1 Ratio: Use in Distinguishing between Infection and Flare-Up in Febrile Patients with Systemic Lupus Erythematosus. The C4d/CR1 ratio is a simple and quickly determinable biomarker that enables the differentiation between infection and flare-up in febrile SLE patients at initial evaluation. PubMed, Biomed Res Int.

Gastrointestinal

The Gastrointestinal System. People with lupus may be affected by problems in any area of the GI system, including the surrounding organs such as the liver, the pancreas, the bile ducts, and the gallbladder. Lupus Foundation of America.

Effect of early eradication therapy on systemic lupus erythematosus (SLE) risk in patients with Helicobacter pylori (HP) infection: a nationwide population-based cohort study. Early HP eradication could significantly reduce SLE risk, especially in the first three-year follow-up. PubMed, Lupus, 2020 May 7:961203320923393.

Case Report: Treatment of colon cancer in a patient with systemic lupus erythematosus. This report provides an effective way to diagnose colon cancer in patients with systemic lupus erythematous and illustrates a successful therapy strategy for this complex medical condition. PubMed, BMC Cancer. (Also see Cancer)

Hearing

Effect of systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) on sudden sensorineural hearing loss (SSNHL). Comorbid SLE or RA may negatively affect the prognosis of SSNHL. PubMed, Laryngoscope, 12/04/2019. (Also see Symptoms and Complications of Rheumatoid Arthritis and Autoimmune Ear Diseases)

Heart Disease

Systemic lupus erythematosus; stroke and myocardial infarction (MI) risk: a systematic review and meta-analysis. Overall, patients with SLE were identified to have a twofold to threefold higher risk of stroke and MI. PubMed, Clin Lab, RMD Open, 2020 Sep;6(2):e001247.

Prevalence of antiphospholipid antibodies in patients with overt myocardial dysfunction in systemic lupus erythematosus. A case-control study. This study did not find any significant association of anti-phospholipid antibodies with overt myocardial dysfunction in patients of SLE. PubMed, Lupus, 2020 Aug 4;961203320947784. (Also see Antiphospholipid Syndrome)

Differences in HDL-Cholesterol Efflux Capacity (CEC) Between Patients with Systemic Lupus Erythematosus (SLE) and Rheumatoid Arthritis (RA). RA patients have a more pro-atherogenic lipid pattern compared to those with SLE. However, CEC seems to be more damaged in SLE than in RA patients. PubMed, Arthritis Care Res (Hoboken), 08/08/2020. (Also see Symptoms and Complications of Rheumatoid Arthritis)

Left Ventricular (LV) Systolic Function in Patients With Systemic Lupus Erythematosus (SLE) and Its Association With Cardiovascular Events. In patients with SLE, LV systolic function as measured by LV global longitudinal strain is significantly impaired and associated with cardiovascular events. PubMed, J Am Soc Echocardiogr, 2020 Jul 1;S0894-7317(20)30230-3.

A Population-based Study of Risk Factors for Heart Failure in Pediatric and Adult-onset Systemic Lupus Erythematosus (SLE. Hypertension remains an important traditional cardiovascular risk factor across all ages and should be managed aggressively even in younger patients with SLE. Seminars in Arthritis and Rheumatism, 05/03/2020.

Maternal hypertensive disorders (HDP) in systemic lupus erythematosus (SLE) pregnancy and future cardiovascular outcomes. In women with and without SLE, HDP were associated with a three-fold higher rate of hypertension. PubMed, Arthritis Care Res (Hoboken), 01/31/2020.

Systemic lupus erythematosus (SLE) is a risk factor for atrial fibrillation (AF): a nationwide, population-based study. Development of AF in patients with SLE was associated with increased mortality. PubMed, Clin Exp Rheumatol, 2019 Nov-Dec;37(6):1019-1025.

Heart failure (HF) risk in systemic lupus erythematosus (SLE) compared to diabetes mellitus (DM) and general medicaid patients. Further investigation into the biologic mechanism of HF among SLE compared to non-SLE and DM patients may shed light on the findings of this study. PubMed, Semin Arthritis Rheum, 06/11/2019. (Also see Symptoms and Complications of Diabetes)

Metabolic syndrome (MetS) in patients with systemic lupus erythematosus: Association with disease activity, disease damage and age. The frequency of MetS was not significantly different from that of the control group, but almost half of the patients were found with this syndrome. PubMed, Int J Rheum Dis, 04/03/2018.

Novel gene variants associated with cardiovascular disease (CVD) in systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA). The IL19 risk allele was associated with stroke/MI in SLE and RA, but not in the general population, indicating that shared immune pathways may be involved in the CVD pathogenesis in inflammatory rheumatic diseases. PubMed, Ann Rheum Dis, 03/07/2018. (Also see Symptoms and Complications of Rheumatoid Arthritis)

Cardiac complications in rheumatoid arthritis (RA), systemic lupus erythematosus (SLE) and systemic sclerosis (SSc). Since an increased risk of cardiovascular complications applies to many rheumatic diseases, the European League Against Rheumatism (EULAR) has developed a number of recommendations related to the prevention and therapy of cardiovascular events. PubMed, Przegl Lek, 2017;74(4):179-82. (Also see Symptoms and Complications of Rheumatoid Arthritis and Complications with Systemic Scleroderma (SSc) Heart Involvement)

Risk of Myocardial Infarction and Stroke in Newly Diagnosed Systemic Lupus Erythematosus: A General Population-Based Study. Patients with SLE have an increased risk of cardiovascular events, particularly during the first year after diagnosis. PubMed, Arthritis Care Res (Hoboken), 01/27/2017.

Jaccoud's Arthritis

Jaccoud's Arthritis or Jaccoud's arthropathy. A progressive deforming arthropathy of the hands and feet in young adults following recurrent rheumatic fever or systemic lupus erythematosus. Who named it?

Kidney Involvement

Lupus Kidney Disease. The two medical terms for the kidney disease that occurs in systemic lupus erythematosus are lupus nephritis or lupus glomerulonephritis. It is estimated that about one-third of people with lupus will develop nephritis that requires medical evaluation and treatment. Lupus Foundation of America.

Comparison of Clinical Features and Outcomes Between Patients With Early and Delayed Lupus Nephritis Clinical outcomes of early and delayed lupus nephritis are not significantly different. PubMed, BMC Nephrol, 2020 Jul 7;21(1):258.

Interleukin-34 as a marker for subclinical proliferative lupus nephritis (LN). Serum IL-34 may be used as a surrogate marker for early renal affection in silent LN, especially the proliferative type. Sage Journals, Lupus, 04/01/2020. (Also see Interleukins)

Transplant outcomes in kidney recipients with lupus nephritis (LN), and systematic review. Kidney transplantation is an acceptable option in patients with concurrent LN and end-stage renal disease. PubMed, Lupus, 2020 Mar;29(3):248-255. (Also see Kidney Transplant)

Dysregulated Fc gamma receptor IIa-induced cytokine production in dendritic cells (DCs) of lupus nephritis patients. We identified that DCs of lupus nephritis patients show increased Fcγ R-induced IL-1β production, which is another important cytokine that promotes kidney inflammation. PubMed, Clin Exp Immunol, 09/11/2019.

Relationship between interstitial CD34 positive cells and active phase of lupus nephritis. A strong interstitial expression of CD34 was observed in lower activity indices and it could play a protective role in lupus nephritis and could reduce renal activity. PubMed, Eur J Rheumatol, 2018 Oct 10:1-4.

Effect of low and high HDL-C levels on the prognosis of lupus nephritis (LN) patients: a prospective cohort study. Our results demonstrate that high HDL-C levels were associated with increased risk of end–stage renal disease in LN patients with advanced renal dysfunction. PubMed, Lipids Health Dis, 2017 Dec 6;16(1):232.

Recent advances in the understanding of renal inflammation and fibrosis in lupus nephritis. This review discusses recent advances in the understanding of pathogenic mechanisms leading to inflammation and fibrosis of the kidney in lupus nephritis in the context of established standard–of–care and emerging therapies. PubMed, 1000Res, 2017 Jun 13;6:874.

Pathological spectrums and renal prognosis of severe lupus patients with rapidly progressive glomerulonephritis. Creatinine concentrations in the serum (SCr) and the proportion of crescents were the most important risk factors for end-stage renal disease (ESRD). PubMed, Rheumatol Int.

Liver Involvement

Lupus Hepatitis and Autoimmune Hepatitis (Lupoid Hepatitis). We review the liver diseases associated with SLE and discuss the approach to the diagnostic evaluation of these patients. PubMed, Am J Med Sci, 2017 Apr;353(4):329-335.

Lymphoma

The risks of cancer development in systemic lupus erythematosus (SLE) patients: a systematic review and meta–analysis. This comprehensive meta–analysis provides epidemiological evidence supporting the associations between SLE and cancer risk. BMC Journals, Arthritis Research & Therapy, 12/06/2018. (Also see Cancer)

Lupus–related single nucleotide polymorphisms and risk of diffuse large B-cell lymphoma (DLBCL). These data suggest several plausible genetic links between DLBCL and SLE. PubMed, Lupus Sci Med. 2017 Nov 12;4(1):e000187.

Microstomia (Small Mouth)

Microstomia (small mouth) is most often caused by systemic scleroderma. However, it has occasionally been associated with systemic lupus (SLE) or rheumatoid arthritis (RA), or Sjogren's. ISN.

Microstomia (Small Mouth) and Scleroderma
Prevention of Microstomia
Treatments for Microstomia
Microstomia Personal Stories

Miscarriages

Risk factors of systemic lupus erythematosus (SLE) flares during pregnancy. The SLE flares during pregnancy make the difference between an uncomplicated pregnancy and pregnancy with maternal and fetal complications. Knowledge of risk factors leads the best treatment strategies to reduce flares and fetal complications in SLE patients. PubMed, Immunol Res.

Musculoskeletal

Construction of a Frailty Index (FI) as a Novel Health Measure in Systemic Lupus Erythematosus (SLE). The SLICC-FI may be a useful tool for identifying patients with SLE who are most vulnerable to adverse outcomes. PubMed, J Rheumatol, 2020 Jan;47(1):72-81.

The Impact of Systemic Lupus Erythematosus (SLE) on the Risk of Newly Diagnosed Hip Fracture. A General Population-Based Study. Patients with newly diagnosed SLE have a 62% increased risk of hip fractures compared to non-SLE individuals. PubMed, Arthritis Care Res (Hoboken), 11/23/2019.

Usefulness of composite indices in the assessment of joint involvement in systemic lupus erythematosus (SLE) patients: correlation with ultrasonographic score. We suggest the ability of composite indices in detecting SLE joint inflammation and their possible real–life use. PubMed, Lupus, 2019 Feb 11:961203319828527.

Neuropsychiatric

Different patterns of cerebral perfusion in systemic lupus erythematosus (SLE) patients with and without neuropsychiatric (NP) manifestations. Cerebral blood flow measured by noninvasive three-dimensional arterial spin labeling could be a useful biomarker for the diagnosis and disease monitoring of NPSLE and non-NPSLE patients. PubMed, Hum Brain Mapp, 10/24/2019.

Association of Depression With Risk of Incident Systemic Lupus Erythematosus (SLE) in Women Assessed Across 2 Decades. This study contributes to increasing evidence that depression may be associated with increased risk of SLE. PubMed, JAMA Psychiatry, 2018 Dec 1;75(12):1225-1233. (Also see Depression)

Depression and anxiety in systemic lupus erythematosus: The crosstalk between immunological, clinical, and psychosocial factors. Despite also suffering from the pain and disability that accompanies chronic autoimmune disease, the rheumatoid arthritis patients had less anxiety and better relationship scores. PubMed, Medicine (Baltimore). 2018 Jul;97(28):e11376. (Also see Depression)

Pancreatitis

Systemic lupus erythematosus can cause pancreatitis, and those who have psychosis and pleurisy are at increased risk for pancreatitis.

Persistent Lupus Myelitis

Myelitis in systemic lupus erythematosus: clinical characteristics and effect in accrual damage. A single–center experience. Myelitis is associated with a significant increase in accrual damage compared with severe non–neuropsychiatric manifestations. PubMed, Lupus, 2017 Mar;26(3):248-254.

Pulmonary Involvement

Complement alternative pathway activation associated with pulmonary hypertension in lupus nephritis patients. Activation of the complement alternative pathway may be involved in the pathogenesis of pulmonary hypertension in lupus nephritis. PubMed, Lupus, 2019 Jul 11:961203319860192. (Also see Pulmonary Hypertension)

Case Report: Systemic lupus erythematosus (SLE) presenting as severe alveolar hypoventilation and the shrinking lung syndrome (SLS). The interesting features of this case report consist of the presentation of the SLS as an alveolar hypoventilation with a fatal outcome and the presentation of systemic lupus as SLS. PubMed, Rev Mal Respir. (Also see Lung Involvement)

Decreased diffusion capacity on lung function testing in asymptomatic patients with systemic lupus erythematosus (SLE) does not predict future lung disease. A finding of low diffusion capacity in asymptomatic patients with SLE, with or without antiphospholipid syndrome, does not necessarily require further evaluation and imaging and may improve spontaneously over time. PubMed, Lupus.

Pulmonary Issues and Lupus. Inflammation caused by lupus may affect the lungs in many ways, and can involve the membrane lining of the lungs, the lungs themselves, the blood vessels within the lungs, and the diaphragm. Lupus Foundation of America.

Retinal

Retinal Complications in Patients with Systemic Lupus Erythematosus Treated with Antimalarial Drugs. Just over 5% of patients developed antimalarial retinal complications, over an average of 12.8 years, but no cases were detected in the first 5 years of therapy. PubMed, J Rheumatol, 2020 Apr;47(4):553-556. (Also see DMARDs)

Skin

Pathogenesis of skin injury in systemic lupus erythematosus (SLE). Skin deposited IgG is a crucial pathological factor to trigger skin lesions in SLE and blockade of IgG signaling may be effective target against skin injury of SLE. PubMed, Curr Rheumatol Rep, 2018 Feb 21;20(2):5.

Sleep Disorders

Anemia Linked to Restless Legs Syndrome in Lupus. Anemia could increase the prevalence of restless legs syndrome (RLS) in patients with lupus. Modern Medicine Network.

Thrombocytopenia

Profiling of non-criteria antiphospholipid antibodies in patients with systemic lupus erythematosus (SLE): differentiation of thrombotic SLE patients and risk of recurrence of thrombosis. Profiling of antiphospholipid antibodies by line immunoassay can differentiate SLE patients with thrombosis from SLE patients without thrombosis. PubMed, Lupus, 2020 Mar 4:961203320909952. (Also see Antibodies in Lupus)

Thrombotic thrombocytopenic purpura (TTP) as the first presentation in systemic lupus erythematosus (SLE). Coexistence of TTP with systemic lupus erythematosus (SLE) is extremely rare, and the emergence of the full manifestation of SLE starting with TTP is even rarer. PubMed, Reumatologia, 2018;56(4):268-270.

Thyroid

The Thyroid-Lupus Relationship. Lupus patients should be tested for signs of abnormal thyroid activity, including thyroid antibody levels, which may show up earlier than other features of thyroid disease. Lupus Foundation of America.

Tuberculosis

Active tuberculosis (TB) in patients with systemic lupus erythematosus (SLE) from Southern China: a retrospective study. TB infection is a common comorbidity in patients with SLE and co–infection with TB and other pathogens is not rare. PubMed, Clin Rheumatol, 2019 Feb;38(2):535-543

Vascular Involvement

Complement deposition, C4d, on platelets is associated with vascular events in systemic lupus erythematosus (SLE). PC4d+ is associated with vascular events in SLE, independently of traditional and SLE-associated risk factors. PubMed, Rheumatology (Oxford), 04/07/2020.

Septic shock among patients with systemic lupus erythematosus (SLE): short and long–term outcome. Analysis of a French nationwide database. Septic shock is a frequent and severe complication among SLE patients even if it is not associated with more healthcare use than another episode of same severity. PubMed, J Infect, 04/08/2019.

Endothelial dysfunction in early systemic lupus erythematosus patients and controls without previous cardiovascular events. A high rate of endothelial dysfunction and vascular stiffness occurs in early lupus patients without cardiovascular risk factors and disease. PubMed, Arthritis Care Res (Hoboken), 12/15/2017.

Case Report: Mesenteric Vasculitis in a Case of Systemic Lupus Erythematosus. We present a case of mesentric vasculitis with systemic lupus erythematosus who relapsed after high dose steroids but achieved subsequent remission after starting pulse cyclophosphamide therapy. PubMed, J Assoc Physicians India. (Also see Vasculitis)

Case Report: Successful treatment of cerebral large vessel vasculitis in systemic lupus erythematosus with intravenous pulse cyclophosphamide. Here, we present a rare case of cerebral large vessel vasculitis treated successfully with a clear visual presentation. PubMed, Lupus. (Also see Vasculitis and Immunosuppressants)

Lupus Complications. Lupus may lead to blood problems, including anemia and increased risk of bleeding or blood clotting. It can also cause inflammation of the blood vessels (vasculitis). Mayo Clinic.

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