Clinical manifestations & diagnosis of systemic lupus erythematosus (SLE) in adults

Clinical manifestations & diagnosis of systemic lupus erythematosus (SLE) in adults

 

Clinical manifestations & diagnosis of systemic lupus erythematosus (SLE) in adults

 

SLE: a heterogeneous autoimmune disease showing a wide range of clinical & serologic manifestations which can affect mostly any systemic organ. Disease course is usually marked by remission & relapse that vary from mild to severe course. Majority of clinical manifestations of SLE involve:

(1)  Constitutional Sms: fatigue, fever, & weight loss, seen in most ptns.  

(2)  Arthritis & arthralgias seen in > 90 % of lupus ptns usually one of the earliest manifestations. Many ptns may show skin & mucous membrane lesions.  

(3)  Cardiac disease is commonly seen involving the pericardium, myocardium, valves, conduction tree, & coronaries. Vascular lesions include Raynaud phenomenon, vasculitis, & thromboembolic disease, can be also seen.  

(4)  Renal involvement is clinically evident in almost 50 % of ptns with significant morbidity & mortality.  

(5)  SLE-related GIT disorders can involve almost all parts along the GIT. However, majority of Sms may be attributed to medication SE or to infection.  

(6)  Pulmonary diseases: pleuritis (with/without effusion), pneumonitis, interstitial lung disease, PH, shrinking lung syndrome, & alveolar haemorrhage.  

(7)  Neuropsychiatric involvement: cognitive dysfunction, organic brain syndrome, delirium, psychosis, seizures, headache, and/or peripheral neuropathies.  

(8)  Hematologic diseases: affecting all 3 blood cell lines: anaemia, leukopenia, & thrombocytopenia. Lymphadenopathy & splenomegaly can also be seen.  

 

Proper evaluation of SLE requires a thorough history & physical exam with selected lab testing to recognize major features of SLE. In medical history & physical examination, a specific attention should be paid to the following:

1)    Photosensitivity: malar rash or discoid lesion.

2)    Painless oral/nasal ulcerations

3)    Hair loss: patchy or frontal/peripheral

4)    Raynaud phenomenon

5)    Joint pain/swelling, migratory or symmetrical

6)    Sms of serositis/pericarditis

 

We should also ask about drug-induced lupus (e.g., hydralazine). We obtain a CBC & differential + SCr + urinalysis in all ptns suspected of SLE. Moreover, selected lab testing support Dgx of SLE that include: (ANA; and if +ve, other specific auto-AB, e.g., anti-dsDNA & anti-Sm), antiphospholipid AB, C3 & C4 or CH50 levels, ESR and/or CRP, & urine protein-to-Cr ratio. Additional labs: diagnostic imaging or biopsy of an involved organ, may be included. Classification have been introduced for study workshop.

 

Dgx of SLE is primarily relied upon a judged experienced physician recognizing the major constellations of Sm & Sn in the setting of supportive serologic results after excluding other diagnoses. In view of the great variability in expression & severity of SLE, Dgx of SLE can be challenging, and referral to an experienced rheumatologist might be appropriate. Lack of the "diagnostic criteria," the suggested approach to the SLE Dgx is as follows:

(1)  Definite SLE: After excluding alternate Dgx, we diagnose SLE in ptn fulfilling the 1997 American College of Rheumatology (ACR) criteria or 2012 Systemic Lupus International Collaborating Clinics (SLICC) classification criteria. By time, the way in which the 2019 European League Against Rheumatism (EULAR)/ACR classification criteria eventually fit within this schema will become clearer.  

(2)  Probable SLE: ptns didn’t not fulfil the classification criteria but we still diagnose the disease. These include ptns presenting with an inadequate number of ACR or SLICC criteria or those having other SLE manifestations that’s not included in either classification criteria.  

(3)  Loose Dgx of SLE in ptn with 2-3 of the ACR or SLICC criteria & at least one other feature that may be associated with but not specific for SLE including:

1.    Optic neuritis, aseptic meningitis

2.    Glomerular haematuria

3.    Pneumonitis, pulmonary hemorrhage, or PH, interstitial lung disease

4.    Myocarditis, verrucous endocarditis (Libman-Sacks endocarditis)

5.    Abdominal vasculitis.

6.    Raynaud phenom.

7.    Raised acute phase reactant (e.g., ESR & CRP)

(4)  Possible SLE: a possible Dgx in individuals having only one of the ACR/SLICC criteria, in addition to at least 1 or 2 of the uncommon features.  

(5)  Undifferentiated connective tissue disorder: ptns having fewer features related to SLE may be classified as undifferentiated connective tissue disease (UCTD), a term describing Sn & Sm suggestive of a systemic autoimmune disease but NOT meeting ACR criteria for SLE or another defined CT disease.  

(6)  ANA-negative SLE: < 5 % of SLE ptns are ANA negative by indirect IF. Frequency of ANA-negative SLE is lower in ptns seen at an early stage of the disease. Moreover, SLE ptns with longstanding disease and/or have already treated may lose ANA reactivity and become serologically negative by time. DD of SLE is wide including the connective & other autoimmune disorders.