Test: SLE - NEET PG MCQ

The image depicts an erythematous rash that spares the nasolabial folds, which, along with the clinical history, suggests a diagnosis of SLE.

• Choice B indicates a heliotrope rash affecting the upper eyelid and proximal muscle weakness.
• Choice C manifests as grey-brown patches, typically found on the face, including the cheeks, bridge of the nose, forehead, chin, and above the upper lip.
• These patches may also appear on sun-exposed areas of the body, such as the forearms and neck.
• Choice D shows redness that can gradually extend beyond the nose and cheeks to the forehead and chin, accompanied by flushing, visible blood vessels, and acne-like eruptions.

Clinicai abnormalities such as palpable purpura, pulmonary infiltrates, microscopic haematuria, mononeuritis multiplex, and glomerulonephritis presenting in combination should indicate a potential diagnosis of vasculitis. Therefore, in the evaluation of these patients, testing for ANCA is essential.

• ANCA are antibodies that target proteins found in the cytoplasmic granules of neutrophils and monocytes.
• They are commonly detected in individuals with:
  • Granulomatosis with angitis
  • Microscopic angitis
  • Churg-Strauss syndrome

A definitive diagnosis is established through the biopsy results of the affected organs. If conditions such as PAN, Takayasu arteritis, or CNS vasculitis are suspected, an arteriogram of the potentially involved organs should be conducted.

As per the SLICC criteria, the primary lesion observed in SLE is synovitis rather than arthritis. The clinical features of SLE include:

• Skin: acute/subacute cutaneous LE
• Oral ulcers
• Alopecia
• Synovitis
• Renal lesions: proteinuria, RBC casts, confirmed by biopsy
• Neurological symptoms: seizures, psychosis, mono-neuritis, myelitis, peripheral neuropathy, cranial nerve involvement, confusional state
• Haemolytic anaemia
• Leukopenia/lymphopenia
• Thrombocytopenia

Immunological features include:

• ANA > reference negative value
• Anti-Sm
• Anti-dsDNA
• Antiphospholipid antibody
• Low serum complement
• Positive direct Coombs test

To meet the criteria, there must be a total of four features, which should include more than one clinical feature and one immunological feature, or biopsy-proven lupus nephritis along with anti-dsDNA antibodies or ANA.

• Diseases associated with antibodies and immune complexes:
  • Autoimmune haemolytic anaemia
  • Autoimmune thrombocytopenia
  • Myasthenia gravis
  • Graves' disease
  • Goodpasture syndrome
  • Systemic lupus erythematosus (SLE)
• Diseases mediated by T cells:
  • Type 1 diabetes mellitus
  • Multiple sclerosis
  • Rheumatoid arthritis
  • Systemic sclerosis
  • Sjögren's syndrome
• Diseases caused by autoimmunity or reactions to microbial antigens:
  • Inflammatory bowel disease (Crohn's disease, ulcerative colitis)
  • Inflammatory myopathies

• Anti-Rho or SS-A antibodies are observed in SLE and Sjögren syndrome. They can also be passed through the placenta, leading to neonatal lupus, which manifests as complete heart block.
• Mixed connective tissue disorder is linked with U, R.N.P. (Ribo Nucleo protein antibody).

The diagnosis is determined by:

• Joint pain
• Fever accompanied by a rash
• Photosensitivity

Rheumatoid arthritis typically affects the small joints symmetrically and is characterised by the absence of a rash.
The diagnostic criteria for SLE are as follows:

• Rash Fixed erythema, over the Malar Eminences
• Erythematous circular raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur
• Photosensitivity Exposure to UV light causes rash
• Oral ulcers Includes oral and nasopharyngeal ulcers
• Synovitis/Non-erosive arthritis of two or more peripheral joints
• Serositis: Pleuritis or pericarditis documented by ECG or rub or evidence of effusion
• Renal disorder Proteinuria >0.5 g/d or 3+, or cellular casts
• Neurologic disorder Seizures or psychosis without other causes
• Hematologic disorder Hemolytic anemia or leukopenia
• Immunological criteria like positive CRP, low C3, ELISA cardiolipin antibody
• ANA positivity

High ESR with normal CRP

• SLE
• Giant cell arteritis
• Multiple myeloma
• Leukemia
• Ulcerative colitis

The inquiry pertains not to systemic lupus erythematosus but rather to subacute cutaneous lupus erythematosus. A majority of individuals with subacute cutaneous lupus erythematosus (SCLE) display a positive result for antinuclear antibodies (ANA) as well as Anti-Ro (SS-A) autoantibodies. The presence of Anti-Ro antibodies is observed in:

• Annular SCLE
• Papulosquamous SCLE
• SCLE associated with vasculitis, Sjögren syndrome, or C2d deficiency
• Mothers of infants diagnosed with neonatal lupus erythematosus (NLE)
• Drug-induced SCLE

Libman-Sacks endocarditis, also referred to as verrucous, marantic, or nonbacterial thrombotic endocarditis, is the most distinctive cardiac manifestation of the autoimmune disorder systemic lupus erythematosus. The valve most frequently affected is the mitral valve, followed by the aortic valve, although all valves can be involved.

The clinical manifestations of localized scleroderma include three distinct morphologic types: morphea, generalized morphea, and linear scleroderma.

• The latter is noted to have a greater prevalence of antinuclear antibodies.
• It has also been linked to antibodies against single-stranded DNA (ssDNA).

The positivity rates for the ANA screening test (on Hep-2 cells) are as follows:

• Mixed connective tissue disease: 100%
• Drug-induced lupus erythematosus: 100%
• Systemic lupus erythematosus: 95%-100%
• Sjögren syndrome: 80%
• Scleroderma: 60%-95%
• Polymyositis-dermatomyositis: 49%-74%
• Rheumatoid arthritis: 40%-60%
• Normal: Less than 4%

• Anti ds-DNA antibodies are linked to a higher risk of nephritis in instances of SLE.
• Anti-centromere antibodies are connected to limited scleroderma.
• Antibodies targeting Ribosomal-P antigens are associated with CNS lupus.
• Antibodies against tRNA synthetase are related to polymyositis with interstitial lung disease.

Fatigue and effusions in serous cavities are characteristics of myxedema heart. Myxedema can result in carpal tunnel syndrome and is linked to macrocytic anaemia. In systemic lupus erythematosus (SLE), the type of anaemia present is autoimmune haemolytic anaemia, which is normocytic normochromic, whereas the anaemia referred to in the question is macrocytic. Notably, carpal tunnel syndrome is not included in the features associated with SLE. The typical presentation of a triad consisting of fever, joint pain, and rash in a woman of childbearing age should raise suspicion for SLE.

The butterfly rash associated with Systemic Lupus Erythematosus (SLE) typically affects several facial areas. However, it generally does not cover the nasolabial fold, which is the area between the nose and the corners of the mouth.
Here are the affected areas:

• Cheeks
• Lower eyelids
• Bridge of nose

In summary, the nasolabial fold remains unaffected by the butterfly rash in SLE.

Lupus anticoagulant is an immunoglobulin that targets phospholipids and proteins linked to the cell membrane. It is a mislabelled term, as it functions as a pro-thrombotic agent. Specifically, lupus anticoagulant antibodies in living organisms result in an increased risk of abnormal blood clotting. This can result in venous and arterial thrombosis in the uterus, which may lead to repeated miscarriages.

Pericarditis is the most prevalent cardiac issue among patients with systemic lupus erythematosus (SLE), although damage to the valves, myocardium, and coronary vessels can also happen.

Historically, cardiac complications were often severe and life-threatening, frequently resulting in death.

Echocardiography is a sensitive and specific method for identifying cardiac irregularities, especially:

• mild pericarditis
• valvular lesions
• myocardial dysfunction

Thus, it is advisable for echocardiography to be conducted regularly in SLE patients.

Vascular occlusion, particularly in the coronary arteries, may arise from:

• vasculitis
• premature atherosclerosis
• anti-phospholipid antibodies linked to SLE

Premature atherosclerosis is the leading cause of coronary artery disease (CAD) among individuals with SLE.

Lupus nephritis is the most common cause of death in children with systemic lupus erythematosus (SLE). This condition primarily affects the kidneys, leading to serious complications. Here are some key points:

• Kidney Damage: Lupus nephritis causes inflammation and damage to the kidneys, affecting their ability to filter waste.
• Symptoms: Patients may experience swelling, high blood pressure, and changes in urine output.
• Treatment: Management often involves medications to reduce inflammation and suppress the immune system.

Due to these serious complications, lupus nephritis significantly increases the risk of mortality in affected children.

Pediatric lupus accounts for 20% of the overall cases of SLE. Children typically exhibit a more acute presentation of the illness and display more frequent involvement of the hematologic and central nervous systems at diagnosis when compared to adults with SLE.

• Almost all children require corticosteroids throughout their illness.
• A significant number are treated with immunosuppressive medications.

Mortality rates for pediatric SLE are consistently higher than those for adult-onset SLE. When comparing pediatric SLE directly with adult SLE, inflammatory rashes, including the characteristic malar erythema, occur much more frequently in children than in adults. Isolated discoid lupus erythematosus (DLE) is rare in childhood. As option 'a' refers to skin pigmentation and not skin rash, it has been excluded.

• Gender disparities in SLE:
  • In the first decade of life: 4:1
  • In the second decade: 9:1
  • Decreases to 5:1 in cases of SLE that begin after the age of 50.

Anti-phospholipid antibodies are frequently, though not always, linked to negative obstetric outcomes, such as:

• First trimester miscarriage
• Mid-trimester and later fetal loss
• Intrauterine death
• Stillbirth

Nevertheless, the likelihood of pregnancy loss is highest during the mid-trimester. The diagnostic criteria for APLS consist of:

• Unexplained deaths of a morphologically normal fetus (confirmed by ultrasound or direct examination of the fetus) at or after the 10^th week of gestation.
• Three or more consecutive unexplained spontaneous abortions prior to the 10^th week of gestation, excluding maternal anatomical or hormonal abnormalities, as well as paternal and maternal chromosomal factors.
• At least one premature birth of a morphologically normal neonate before the 34^th week of gestation due to eclampsia or severe pre-eclampsia, as defined by standard criteria, or recognised signs of placental insufficiency.

Most frequently, the presentation of SLE includes arthralgia or myalgia in 90% of patients. Non-erosive polyarthritis occurs in 60% of individuals with the condition. The most prevalent haematological manifestation of SLE is anaemia. The most common neurological manifestation is a cognitive disorder. In terms of cardiopulmonary presentation, pleurisy is more typical than Libman-Sacks endocarditis.

• Lupus cerebritis and lupus nephritis also show a positive response to steroids.
• No specific treatment is recommended for Libman-Sacks endocarditis.
• Intervention may be necessary for haemodynamically significant valvular dysfunction.

• vasodilators,
• beta blockers,
• diuretics,
• and digoxin.

Cerebritis, nephritis, and episcleritis are uncommon manifestations of drug-induced lupus erythematosus (LE), while skin-related symptoms are more frequently observed. The typical age range is between 50 and 70 years, with symptoms potentially arising up to two years after drug exposure.
Comparison of SLE and Drug-Induced Lupus Erythematosus Findings

No specific treatment is recommended for Libman-Sacks endocarditis. Management of heart failure resulting from valvular dysfunction should follow standard guidelines. Possible medications may consist of:

• Vasodilators
• Beta blockers
• Diuretics
• Digoxin

Seizures are already recognised to occur in 14-25% of individuals with lupus, in contrast to 0.5-1% in the general populace. They may arise due to:

• cerebral vasculitis (ischaemic or haemorrhagic manifestations)
• cardiac embolism
• opportunistic infections
• drug toxicity
• associated metabolic imbalances

Less frequent neurological syndromes that may present in patients known to have SLE include:

• movement disorders (chorea, ataxia, Parkinsonism)
• pseudotumour cerebri
• venous sinus thrombosis

Cerebrospinal fluid (CSF) can indicate heightened central nervous system (CNS) lupus activity by demonstrating raised levels of white cells, protein, immunoglobulin synthesis, or absolute immunoglobulin G (IgG). Anti-neuronal nuclear antibodies can be somewhat helpful in confirming CNS disease when tested on CSF, although these are less specific or sensitive compared to a serum test. The aquaporin 4 antibody is utilised for the diagnosis of Devic's disease (neuromyelitis optica), which is a variant of multiple sclerosis.

• SLE
• Inflammatory bowel disease
• Rheumatic fever
• Behçet's disease
• Henoch-Schönlein purpura

• Prolonged prothrombin time is observed in cases of chronic liver disease.
• Antiphospholipid syndrome triggers the intrinsic clotting system, resulting in an extended aPTT.