Question

What is Systemic Lupus Erythematosus (SLE)? What are the clinical manifestations of SLE?

Answer 1

ANSWER:- Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that can affect almost any organ system. Its presentation and course are highly variable, ranging from indolent to fulminant.

The classic presentation of a triad of fever, joint pain, and rash in a woman of childbearing age should prompt investigation into the diagnosis of SLE.However, patients may present with any of the following types of manifestations :-

1. Constitutional : Fatigue, fever, arthralgia, and weight changes are the most common symptoms in new cases or recurrent active SLE flares.

2. Musculoskeletal : Joint pain is one of the most common reasons for the initial clinical presentation of patients with SLE. Arthralgia, myalgia, and frank arthritis may involve the small joints of the hands, wrists, and knees (usually symmetrical, polyarticular). In contrast to rheumatoid arthritis, SLE arthritis or arthralgia may be asymmetrical, with pain that is disproportionate to swelling.

3. Dermatologic : Cutaneous manifestations of SLE include 3 American College of Rheumatology (ACR) lupus diagnostic criteria: malar rash, photosensitivity, and discoid lupus.

4. Renal : The kidney is the most commonly involved visceral organ in SLE. Although only approximately 50% of patients with SLE develop clinically evident renal disease, biopsy studies demonstrate some degree of renal involvement in most patients.

5. Neuropsychiatric : The CNS lupus nomenclature has been revised to catalog many manifestations.Because of the difficulty distinguishing causal SLE associations with some neurologic symptoms, only seizure and psychosis were typically included in the diagnostic criteria. Seizures related to SLE may be generalized or partial and may precipitate status epilepticus. Psychosis may manifest as paranoia or hallucinations.

However, the American College of Rheumatology (ACR) created standardized case definitions and diagnostic testing recommendations for 19 neuropsychiatric syndromes in SLE, including seizures/seizure disorders and psychosis.The remainder of the neuropsychiatric syndromes are as follows:

• Acute confusional state

• Acute inflammatory demyelinating polyradiculoneuropathy (Guillain-Barre syndrome)

• Anxiety disorder

• Aseptic meningitis

• Autonomic disorder

• Cerebrovascular disease

• Cognitive dysfunction

• Cranial neuropathy

• Demyelinating syndrome

• Headache

• Mononeuropathy (single/multiplex)

• Mood disorders

• Movement disorder (chorea)

• Myasthenia gravis

• Myelopathy

• Plexopathy

• Polyneuropathy

6. Pulmonary : Pulmonary features of SLE may manifest acutely or indolently, representing a spectrum of SLE complications. SLE may lead to multiple pulmonary complications, including pleurisy, pleural effusion, pneumonitis, pulmonary hypertension, and interstitial lung disease.

7. Gastrointestinal : In general, gastrointestinal symptoms secondary to SLE are less common than adverse effects of medication or nonspecific complaints. Special consideration should be given to infectious causes (bacterial, viral [eg, CMV]), because of immunosuppression. Nausea and dyspepsia are common symptoms in patients with active SLE and are sometimes difficult to correlate with objective evidence of gastrointestinal involvement. Peptic ulcer disease is a common complication, especially in SLE patients treated with nonsteroidal anti-inflammatory agents (NSAIDs) and glucocorticoids.

8. Cardiac : Heart failure or chest pain must be carefully assessed in patients with SLE. Pericarditis is the most common cardiac feature of SLE, manifesting as positional chest pain that is often relieved when the patient leans forward. Myocarditis may occur in SLE with heart failure symptoms. Pulmonary hypertension may present with indolent chest pain or dyspnea.

9. Hematological : A history of multiple cytopenias such as leukopenia, lymphopenia, anemia, or thrombocytopenia may suggest SLE, among other etiologies, such as medication-related cytopenias. Leukopenia and, more specifically, lymphopenia are common in SLE; this, coupled with immunosuppression, may predispose persons with SLE to frequent infections.