Systemic lupus erythematosus history and symptoms

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Raviteja Guddeti, M.B.B.S. [2]

Overview

Systemic lupus erythematosus (SLE) one of several diseases known as the great imitator because its symptoms vary so widely it often mimics or is mistaken for other illnesses, and because the symptoms come and go unpredictably. Diagnosis can be elusive, with patients sometimes suffering unexplained symptoms and untreated SLE for years.

History and symptoms

Gastrointestinal involvement:

Organ system	Disease	Sympton
Gastrointestinal involvement	Dysphagia	Retrosternal chest pain Heartburn Regurgitation Odynophagia
	peptic ulcer disease	Epigastric pain Food-provoked epigastric discomfort and fullness Early satiety Nausea
	Intestinal pseudo-obstruction	Abdominal pain Bloating Distension
	Protein-losing enteropathy	Profound edema Hypoalbuminemia Severe diarrhea
	Acute pancreatitis	Severe persistent epigastric pain often radiating to the back
	Mesenteric vasculitis	Abdominal pain Food aversion Weight loss Nausea Vomiting Diarrhea Due to chronic mesenteric ischemia
	Primary peritonitis	Abdominal bloating or distention Nausea and vomiting Diarrhea Constipation or the inability to pass gas Anorexia
Pulmonary involvement	Pleural disease	Cough Dyspnea Fever
	Acute pneumonitis	Fever Cough (sometimes with hemoptysis) Dyspnea/ physical examination reveals tachypnea, tachycardia, basilar crackles (may be late inspiratory), and hypoxemia
	Pulmonary hemorrhage	Dyspnea Cough Hemoptysis
	Interstitial lung disease	May be asymptomatic Chronic nonproductive cough Dyspnea Decreased exercise tolerance
	Thromboembolic disease	Dyspnea Sharp chest pain that may become worse with deep breathing or coughing
	Pulmonary hypertension	Dyspnea Palpitations Fatigue Impaired exercise tolerance Weakness Syncope Edema Increased abdominal girth/ phE=pulmonary hypertension (loud second heart sound) or cor pulmonale (eg, peripheral edema, ascites, hepatomegaly)/
	Shrinking lung	Dyspnea Pleuritic chest pain (episodic)
Cardiac involvement	Valvular disease	most often valve thickening, nodules, and regurgitation, and less frequently vegetations or stenotic lesions, is noted echocardiographically, and the presence of valvular lesions may increase the risk of serious complications
	Nonbacterial thrombotic endocarditis (Libman-Sacks, verrucous endocarditis)	may occur in patients with SLE and is associated with antiphospholipid antibodies (aPL).
	Pericardial disease	is noted in approximately one-half of patients with SLE at some time in the course of their disease. Diagnostic pericardiocentesis is suggested for those in whom purulent pericarditis is suspected and for those who do not respond to treatment with nonsteroidal antiinflammatory drugs (NSAIDs) and/or glucocorticoids.
	Acute pericarditis	Symptomatic often responds to an NSAID; those who do not tolerate or cannot take NSAIDs may use prednisone (0.5 to 1 mg/kg/day in divided doses)
	Myocarditis	in SLE can cause resting tachycardia, cardiomegaly, heart failure, conduction abnormalities, and/or arrhythmias. myocarditis, we suggest initial treatment with high-dose glucocorticoids (Grade 2C). A typical regimen is methylprednisolone 1000 mg intravenously daily for three days.
	Coronary heart disease
Neurological involvement	Cognitive dysfunction
	Stroke	mechanisms are heterogenous in SLE and include arterial and venous thrombosis, cardiogenic embolism, and small vessel infarcts
	Seizures
	Psychosis	Hallucinations visual Auditory
	Headache
	Neuropathies
Genitourinary	Nephrotic syndrome	Hypertension Peripheral edema Foamy urine Weight gain
Musculoskeletal	arthritis	arthralgias effusions Decreased range of motion of both small and large joints Morning stiffness
	avascular necrosis	Asymptomatic Mild to moderate pain in groin and lower abdoman
	bone fragility fractures
	secondary pain amplification
	Avascular necrosis	can occur in patients treated with corticosteroids
	Osteoporosis	Loss of height Sudden back pain
Skin disorder

Common initial and chronic complaints are fever, malaise, joint pains, myalgias and fatigue. Because they are so often seen with other diseases, these signs and symptoms are not part of the diagnostic criteria for SLE. When occurring in conjunction with other signs and symptoms, however, they are considered suggestive.

Most Common Symptoms

• Joint pain or joint swelling
• Skin rash
• Malaise or fatigue

Complete List Symptoms to look out for

• Abdominal pain
• Fatigue
• Keratoconjunctivitis sicca
• Leukopenia
• Pericarditis
• Pleural effusion
• Pleuritic chest pain
• Pneumonitis
• Polyarthritis / polyarthralgia
• Renal disease
• Renal vasculitis
• Seizures
• Stroke
• Thrombocytopenia
• Weight loss

Warning Signs of a Flare

• Increased fatigue
• Pain
• Rash
• Fever
• Abdominal discomfort
• Headache
• Dizziness

Common Symptoms Explained

Dermatological manifestations

As many as 30% of patients present with some dermatological symptoms (and 65% suffer such symptoms at some point), with 30% to 50% suffering from the classic malar rash (or butterfly rash) associated with the disease. Patients may present with discoid lupus (thick, red scaly patches on the skin). Alopecia, mouth, nasal, and vaginal ulcers, and lesions on the skin are also possible manifestations.

Musculoskeletal manifestations

Patients most often seek medical attention for joint pain, with small joints of the hand and wrist usually affected, although any joint is at risk. The Lupus Foundation of America "estimates that 95 percent of lupus cases involve achy joints. Unlike rheumatoid arthritis, SLE arthropathy is not usually destructive of bone, however, deformities caused by the disease may become irreversible in as many as 20% of patients.This small percentage may experience "damage to tendons or joint coverings in the hand" which leads to "deformity of the finger joint".

Hematological manifestations

Anemia and iron deficiency may develop in as many as half of patients. Low platelet and white blood cell counts may be due to the disease or a side-effect of pharmacological treatment. Patients may have an association with antiphospholipid antibody syndrome (a thrombotic disorder) where autoantibodies to phospholipids are present in the patient's serum. Abnormalities associated with antiphospholipid antibody syndrome include a paradoxical prolonged PTT (which usually occurs in hemorrhagic disorders) and a positive test for antiphospholipid antibodies; the combination of such findings have earned the term "lupus anticoagulant positive". Another autoantibody finding in lupus is the anticardiolipin antibody which can cause a false positive test for syphilis.

Cardiac manifestations

Patients may present with inflammation of various parts of the heart, such as pericarditis, myocarditis, and endocarditis. The endocarditis of SLE is characteristically non-infective (Libman-Sacks endocarditis) and involves either the mitral valve or the tricuspid valve. Atherosclerosis also tends to occur more often and advance more rapidly in SLE patients than in the general population.

Pulmonary manifestations

Lung and pleura inflammation can cause pleuritis, pleural effusion, lupus pneumonitis, chronic diffuse interstitial lung disease, pulmonary hypertension, pulmonary emboli, pulmonary hemorrhage.

Hepatic involvement

See autoimmune hepatitis.

Renal involvement

Painless hematuria or proteinuria may often be the only presenting renal symptom. Acute or chronic renal impairment may develop with lupus nephritis, leading to acute or end stage renal failure. Because of early recognition and management of SLE, end stage renal failure occurs in less than 5% of patients. Histologically, a hallmark of SLE is membranous glomerulonephritis with "wire loop" abnormalities. This finding is due to immune complex deposition along the glomerular basement membrane leading to a typical granular appearance in immunofluorescence testing.

Neurological manifestations

About 10% of patients may present with seizures or psychosis. A third may test positive for abnormalities in the cerebrospinal fluid.

T-cell abnormalities

Abnormalities in T cell signaling are associated with SLE, including deficiency in CD45 phosphatase and increased expression of CD40 ligand.

Other rarer manifestations

Lupus gastroenteritis, lupus pancreatitis, lupus cystitis, autoimmune inner ear disease, parasympathetic dysfunction, retinal vasculitis, and lupus mesenteric vasculitis.