Leukopenia is an abnormal reduction in white blood cells. It can result from reduced white blood cell production or increased destruction. The main types of white blood cells are neutrophils, lymphocytes, basophils, monocytes, and eosinophils, which help fight different types of infections. Kostmann syndrome is a rare, congenital neutropenia disorder characterized by lack of mature neutrophils and recurrent bacterial infections. Growth factor therapy increases neutrophil production and aids the body's natural healing process.
Systemic Lupus Erythematosus (SLE) is a multi-gene autoimmune disease caused by a combination of genetic and environmental factors. It is characterized by abnormal immune responses that result in inflammation and damage to various organs. Diagnosis requires meeting 4 out of 11 classification criteria relating to clinical symptoms and blood markers. Management aims to induce remission of acute flares, maintain improvements to suppress symptoms, and prevent organ damage. Treatment choices depend on the severity and potential reversibility of manifestations. The goal is controlling symptoms without cure since complete sustained remission is rare.
Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by autoantibody production and damage to multiple organs. It predominantly affects women of childbearing age. SLE is caused by a combination of genetic and environmental factors. The disease is mediated by autoantibodies that form complexes and damage tissues. Common clinical manifestations include rashes, arthritis, kidney inflammation, and neurological and cardiac involvement. Diagnosis is based on identifying clinical and laboratory criteria including autoantibodies. Management involves controlling symptoms with medications like antimalarials and NSAIDs. More severe organ-threatening disease is treated with glucocorticoids and immunosuppressants like cyclophosphamide or mycophenolate
This document discusses autoimmunity and autoimmune diseases. It begins by explaining how the immune system can mistakenly attack self-antigens, leading to autoimmunity. Several organ-specific and systemic autoimmune diseases are described in detail, including how they are mediated by direct cellular damage, stimulating or blocking autoantibodies. Current treatments aim to suppress the immune system generally but do not cure the underlying condition. Experimental therapies discussed include T-cell vaccination, peptide blockade of MHC molecules, and monoclonal antibodies.
- Systemic lupus erythematosus (SLE) is a multi-system autoimmune disease characterized by tissue damage from antibody and complement deposition, leading to periods of disease exacerbation and remission.
- It predominantly affects females of childbearing age and is more prevalent in non-whites. Major features include the presence of autoantibodies against nuclear and other antigens, immune complex deposition in organs like the kidney and skin, and depression of serum complement levels during disease flares.
- The disease has genetic, hormonal, and environmental risk factors and results from a loss of self-tolerance leading to hyperactive B and T cells that produce pathogenic autoantibodies against self-ant
Connective tissue diseases share features of immune dysregulation and autoantibody production directed at nuclear components, causing widespread tissue damage. Systemic lupus erythematosus is characterized by arthritis, rashes, kidney involvement and positive ANA and anti-dsDNA antibodies. Systemic sclerosis involves skin thickening from fibrosis, Raynaud's phenomenon, and autoantibodies like anti-Scl-70. Polymyositis and dermatomyositis cause proximal muscle weakness and inflammation with skin lesions in dermatomyositis.
A 73-year-old woman presented with symptoms of weakness, cough, and fever. On examination, she was somnolent with a low-grade fever, rapid heart rate and breathing, low blood pressure, and crackles in her right lung. Based on her symptoms and exam findings meeting two SIRS criteria, the most likely diagnosis is systemic inflammatory response syndrome (SIRS) due to a potential infection such as pneumonia. Sepsis occurs when SIRS is caused by a confirmed or suspected infection and can progress to septic shock, organ dysfunction, and death if not properly treated.
This document discusses different types of hypersensitivity reactions. Type II hypersensitivity involves autoantibodies that stimulate host target cell receptors, causing issues like autoimmune hemolytic anemia or Graves' disease. Type III hypersensitivity involves immune complex deposition that can cause conditions like Raynaud's phenomenon or membranous glomerulonephritis. Type IV delayed hypersensitivity involves T cell-mediated responses to environmental antigens, commonly causing contact dermatitis.
Leukopenia is an abnormal reduction in white blood cells. It can result from reduced white blood cell production or increased destruction. The main types of white blood cells are neutrophils, lymphocytes, basophils, monocytes, and eosinophils, which help fight different types of infections. Kostmann syndrome is a rare, congenital neutropenia disorder characterized by lack of mature neutrophils and recurrent bacterial infections. Growth factor therapy increases neutrophil production and aids the body's natural healing process.
Systemic Lupus Erythematosus (SLE) is a multi-gene autoimmune disease caused by a combination of genetic and environmental factors. It is characterized by abnormal immune responses that result in inflammation and damage to various organs. Diagnosis requires meeting 4 out of 11 classification criteria relating to clinical symptoms and blood markers. Management aims to induce remission of acute flares, maintain improvements to suppress symptoms, and prevent organ damage. Treatment choices depend on the severity and potential reversibility of manifestations. The goal is controlling symptoms without cure since complete sustained remission is rare.
Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by autoantibody production and damage to multiple organs. It predominantly affects women of childbearing age. SLE is caused by a combination of genetic and environmental factors. The disease is mediated by autoantibodies that form complexes and damage tissues. Common clinical manifestations include rashes, arthritis, kidney inflammation, and neurological and cardiac involvement. Diagnosis is based on identifying clinical and laboratory criteria including autoantibodies. Management involves controlling symptoms with medications like antimalarials and NSAIDs. More severe organ-threatening disease is treated with glucocorticoids and immunosuppressants like cyclophosphamide or mycophenolate
This document discusses autoimmunity and autoimmune diseases. It begins by explaining how the immune system can mistakenly attack self-antigens, leading to autoimmunity. Several organ-specific and systemic autoimmune diseases are described in detail, including how they are mediated by direct cellular damage, stimulating or blocking autoantibodies. Current treatments aim to suppress the immune system generally but do not cure the underlying condition. Experimental therapies discussed include T-cell vaccination, peptide blockade of MHC molecules, and monoclonal antibodies.
- Systemic lupus erythematosus (SLE) is a multi-system autoimmune disease characterized by tissue damage from antibody and complement deposition, leading to periods of disease exacerbation and remission.
- It predominantly affects females of childbearing age and is more prevalent in non-whites. Major features include the presence of autoantibodies against nuclear and other antigens, immune complex deposition in organs like the kidney and skin, and depression of serum complement levels during disease flares.
- The disease has genetic, hormonal, and environmental risk factors and results from a loss of self-tolerance leading to hyperactive B and T cells that produce pathogenic autoantibodies against self-ant
Connective tissue diseases share features of immune dysregulation and autoantibody production directed at nuclear components, causing widespread tissue damage. Systemic lupus erythematosus is characterized by arthritis, rashes, kidney involvement and positive ANA and anti-dsDNA antibodies. Systemic sclerosis involves skin thickening from fibrosis, Raynaud's phenomenon, and autoantibodies like anti-Scl-70. Polymyositis and dermatomyositis cause proximal muscle weakness and inflammation with skin lesions in dermatomyositis.
A 73-year-old woman presented with symptoms of weakness, cough, and fever. On examination, she was somnolent with a low-grade fever, rapid heart rate and breathing, low blood pressure, and crackles in her right lung. Based on her symptoms and exam findings meeting two SIRS criteria, the most likely diagnosis is systemic inflammatory response syndrome (SIRS) due to a potential infection such as pneumonia. Sepsis occurs when SIRS is caused by a confirmed or suspected infection and can progress to septic shock, organ dysfunction, and death if not properly treated.
This document discusses different types of hypersensitivity reactions. Type II hypersensitivity involves autoantibodies that stimulate host target cell receptors, causing issues like autoimmune hemolytic anemia or Graves' disease. Type III hypersensitivity involves immune complex deposition that can cause conditions like Raynaud's phenomenon or membranous glomerulonephritis. Type IV delayed hypersensitivity involves T cell-mediated responses to environmental antigens, commonly causing contact dermatitis.
This document discusses lupus nephritis, including its pathophysiology, risk factors, clinical presentation, investigations, classification, treatment, and prognosis. Key points include:
- Lupus nephritis is a serious manifestation of systemic lupus erythematosus caused by autoantibodies forming immune complexes that deposit in the glomeruli.
- It is classified based on renal biopsy findings, with classes III and IV having the highest risk of progressing to end-stage renal disease.
- Treatment involves immunosuppressive induction therapy with corticosteroids and cyclophosphamide or mycophenolate mofetil to rapidly decrease inflammation, followed by long-term maintenance
Sepsis is a life-threatening condition caused by the body's response to infection. It has been defined in various ways over time, with the most recent Sepsis-3 definition describing it as a dysregulated immune response leading to organ dysfunction. Diagnosis involves assessing symptoms, signs of infection and organ dysfunction, along with diagnostic tests. Management involves rapid fluid resuscitation, antibiotics within 1 hour of recognition, vasopressors to maintain blood pressure and organ perfusion, and treatment of the underlying infection in an intensive care unit. Delays in recognition and treatment can increase mortality risk.
Systemic Lupus Erythematosus (SLE) is an autoimmune disease where the immune system attacks its own tissues. It most commonly affects women aged 15-40. The exact causes are unknown but genetics, viruses, sunlight, and some drugs may play a role. Symptoms vary but can include rashes, joint pain, fatigue, and organ inflammation. Diagnosis involves blood tests and potentially biopsies. Treatment focuses on rest, sun protection, medications like NSAIDs, antimalarials, corticosteroids, and immunosuppressants to control disease activity and prevent organ damage. Nursing care aims to manage symptoms, prevent infections, and educate patients.
Systemic Lupus Erythematosus (SLE) is a complex autoimmune disease. The immune system attacks the body’s cell and tissue, resulting in inflammation and tissue damage. SLE can affect any part of the body, but most often harms the heart, joints, skin, lungs, blood vessels, liver, kidney and nervous system.
Over 40 different genes predispose to SLE.
Characterized by remission and exacerbation.
White Blood Cell Disorders can affect neutrophils, eosinophils, basophils and mast cells. Neutropenia is classified by severity based on absolute neutrophil count and risk of infection. Causes include acquired conditions like drugs/infections or congenital disorders. Hypereosinophilic syndrome is a broad condition caused by primary or secondary eosinophilia leading to tissue damage. Diagnosis involves ruling out secondary causes and identifying organ involvement. Treatment depends on etiology and includes steroids, hydroxyurea, interferon-alpha, imatinib or anti-IL-5 antibodies.
Purpura and leukemia are bleeding disorders. Purpura is characterized by bruising of the skin from bleeding in the capillaries and has two main types: idiopathic thrombocytopenic purpura and anaphylactoid purpura. Leukemia is cancer of the blood or bone marrow cells, characterized by abnormal increase of white blood cells. It is classified based on the predominant cell type and maturity into acute or chronic forms of lymphoid or myeloid leukemia. Both disorders involve uncontrolled growth and proliferation of blood cells.
A presentation detailing the symptoms, pathogenicity, factors affecting, diagnosis and treatment of the autoimmune disorder systemic lupus erythematosus
Lupus Nephritis :From Basics To PracticeYasser Matter
This document provides an overview of lupus nephritis (LN), including:
- Epidemiology: Up to 40% of SLE cases involve the kidneys. It predominantly affects women aged 15-45.
- Pathogenesis: Autoantibodies form immune complexes that deposit in the glomerulus, activating complement and attracting inflammatory cells. The location of deposits determines histologic class.
- Diagnosis: Urinalysis, serum creatinine, immunological tests. Renal biopsy is needed to classify LN and guide treatment.
- Treatment involves induction therapy for 3-6 months, then maintenance to prevent chronic kidney disease. Treatment depends on histologic class and response to therapy. The goal
Systemic lupus erythematosus (SLE) is a multi-system autoimmune disease characterized by autoantibody production and organ damage. It predominantly affects women of childbearing age. The disease has an unknown etiology involving genetic, environmental, and immune system factors. Common clinical manifestations include arthritis, rash, oral ulcers, photosensitivity, and involvement of major organ systems like the kidneys, heart, and lungs. Diagnosis is based on clinical criteria and the presence of autoantibodies. Treatment involves managing symptoms, preventing flares, and suppressing the immune system using medications like corticosteroids, antimalarials, azathioprine, and cyclophosphamide. Pro
AUTOIMMUNITY AND AUTO IMMUNE DISEASES.pdfnithyatulasi1
The immune system could go awry and, instead of reacting against foreign antigens, could focus its attack on self-antigens. Paul Ehrlich termed this condition “horror autotoxicus.”
Mechanisms of self-tolerance normally protect an individual from potentially self-reactive lymphocytes, there are failures. They result in an inappropriate response of the immune system against self-components termed autoimmunity.
AUTOIMMUNITY AND ITS ASSOCIATED DISEASES.pdfnithyatulasi1
The immune system could go awry and, instead of reacting against foreign antigens, could focus its attack on self-antigens. Paul Ehrlich termed this condition “horror autotoxicus.”
Mechanisms of self-tolerance normally protect an individual from potentially self-reactive lymphocytes, there are failures. They result in an inappropriate response of the immune system against self-components termed autoimmunity.
Anaesthesia for neurological and neuromuscular disease2Kanika Rustagi
The document discusses various neurological and neuromuscular diseases relevant to anaesthesia including epilepsy, multiple sclerosis, Guillain-Barre syndrome, poliomyelitis, and cerebral palsy. It covers the pathophysiology, clinical features, diagnostic criteria, and anaesthetic considerations for managing patients with these conditions. Key points discussed include preoperative assessment and planning, choice of anaesthetic agents to avoid exacerbating symptoms, special monitoring needs, and postoperative care considerations.
Agranulocytosis is a condition involving a severe decrease in neutrophils in the blood, increasing the risk of infection. It can be caused by decreased neutrophil production due to conditions like aplastic anemia or cancer treatments, or increased neutrophil destruction from infections, autoimmune disorders, or drug reactions. Symptoms include fever, chills, and mouth sores. Diagnosis is made through blood tests showing very low neutrophil counts. Treatment focuses on managing infections with antibiotics, stimulating new neutrophil production with growth factors, and addressing any underlying causes.
This document provides information about systemic lupus erythematosus (SLE). It defines SLE as a multi-system autoimmune disease affecting various organs mediated by autoantibodies and immune complexes. The causes are unknown but may involve genetic, hormonal, and environmental factors. SLE can affect many organ systems like the kidneys, lungs, heart, and nervous system, causing a variety of clinical manifestations. Diagnosis involves evaluating symptoms, medical history, physical exam, and laboratory tests. Treatment depends on disease severity and organ involvement, and may include medications like NSAIDs, antimalarials, corticosteroids, and cytotoxic drugs. Special considerations are given to SLE in pregnancy and neonates.
Systemic Lupus Erythematosus (SLE) is an autoimmune disease that can affect multiple organ systems. It typically affects young women and is characterized by periods of disease flares and remissions. While the exact cause is unknown, genetic and environmental factors likely contribute to abnormal immune system functioning and production of autoantibodies. Common clinical manifestations include malar rash, arthritis, kidney problems, and hematological abnormalities. Diagnosis involves identifying clinical features and detecting autoantibodies such as antinuclear antibodies and anti-dsDNA antibodies. With proper management, 10-year survival rates are over 90%.
Nephritis is a inflammation of kidney .
It is classified into various types like lupus nephritis ,interstitial nephritis , glomerulonephritis ,pyelonephritis.
Lupus nephritis is an inflammation of kidney due to autoimmune disorder named as lupus .
It is inflammation of lower urinary tract .
1. White blood cells (leukocytes) include granulocytes like neutrophils, eosinophils, and basophils which fight infection, and agranulocytes like monocytes and lymphocytes which are involved in immune responses.
2. Leukemia is a cancer of the blood cells characterized by abnormal proliferation of white blood cells. The four main types are acute myeloid leukemia, acute lymphoblastic leukemia, chronic myeloid leukemia, and chronic lymphocytic leukemia.
3. Factors involved in leukemia development include genetic mutations, chromosomal translocations, radiation exposure, certain chemicals, and some viruses. Maintaining overall health can help support white blood cell counts.
This document provides an overview of systemic lupus erythematosus (SLE). It discusses the definition, epidemiology, pathogenesis, diagnosis, clinical manifestations, management, and complications of SLE. The pathogenesis involves genetic susceptibility and environmental triggers leading to abnormal immune responses and autoantibody production. Diagnosis is based on the SLICC classification criteria. Management involves controlling symptoms, preventing organ damage, and treating flares and complications using medications like glucocorticoids, antimalarials, immunosuppressants, and biologics. Life-threatening complications can include renal disease, neurological involvement, hematological abnormalities and vasculitis.
This document discusses lupus nephritis, including its pathophysiology, risk factors, clinical presentation, investigations, classification, treatment, and prognosis. Key points include:
- Lupus nephritis is a serious manifestation of systemic lupus erythematosus caused by autoantibodies forming immune complexes that deposit in the glomeruli.
- It is classified based on renal biopsy findings, with classes III and IV having the highest risk of progressing to end-stage renal disease.
- Treatment involves immunosuppressive induction therapy with corticosteroids and cyclophosphamide or mycophenolate mofetil to rapidly decrease inflammation, followed by long-term maintenance
Sepsis is a life-threatening condition caused by the body's response to infection. It has been defined in various ways over time, with the most recent Sepsis-3 definition describing it as a dysregulated immune response leading to organ dysfunction. Diagnosis involves assessing symptoms, signs of infection and organ dysfunction, along with diagnostic tests. Management involves rapid fluid resuscitation, antibiotics within 1 hour of recognition, vasopressors to maintain blood pressure and organ perfusion, and treatment of the underlying infection in an intensive care unit. Delays in recognition and treatment can increase mortality risk.
Systemic Lupus Erythematosus (SLE) is an autoimmune disease where the immune system attacks its own tissues. It most commonly affects women aged 15-40. The exact causes are unknown but genetics, viruses, sunlight, and some drugs may play a role. Symptoms vary but can include rashes, joint pain, fatigue, and organ inflammation. Diagnosis involves blood tests and potentially biopsies. Treatment focuses on rest, sun protection, medications like NSAIDs, antimalarials, corticosteroids, and immunosuppressants to control disease activity and prevent organ damage. Nursing care aims to manage symptoms, prevent infections, and educate patients.
Systemic Lupus Erythematosus (SLE) is a complex autoimmune disease. The immune system attacks the body’s cell and tissue, resulting in inflammation and tissue damage. SLE can affect any part of the body, but most often harms the heart, joints, skin, lungs, blood vessels, liver, kidney and nervous system.
Over 40 different genes predispose to SLE.
Characterized by remission and exacerbation.
White Blood Cell Disorders can affect neutrophils, eosinophils, basophils and mast cells. Neutropenia is classified by severity based on absolute neutrophil count and risk of infection. Causes include acquired conditions like drugs/infections or congenital disorders. Hypereosinophilic syndrome is a broad condition caused by primary or secondary eosinophilia leading to tissue damage. Diagnosis involves ruling out secondary causes and identifying organ involvement. Treatment depends on etiology and includes steroids, hydroxyurea, interferon-alpha, imatinib or anti-IL-5 antibodies.
Purpura and leukemia are bleeding disorders. Purpura is characterized by bruising of the skin from bleeding in the capillaries and has two main types: idiopathic thrombocytopenic purpura and anaphylactoid purpura. Leukemia is cancer of the blood or bone marrow cells, characterized by abnormal increase of white blood cells. It is classified based on the predominant cell type and maturity into acute or chronic forms of lymphoid or myeloid leukemia. Both disorders involve uncontrolled growth and proliferation of blood cells.
A presentation detailing the symptoms, pathogenicity, factors affecting, diagnosis and treatment of the autoimmune disorder systemic lupus erythematosus
Lupus Nephritis :From Basics To PracticeYasser Matter
This document provides an overview of lupus nephritis (LN), including:
- Epidemiology: Up to 40% of SLE cases involve the kidneys. It predominantly affects women aged 15-45.
- Pathogenesis: Autoantibodies form immune complexes that deposit in the glomerulus, activating complement and attracting inflammatory cells. The location of deposits determines histologic class.
- Diagnosis: Urinalysis, serum creatinine, immunological tests. Renal biopsy is needed to classify LN and guide treatment.
- Treatment involves induction therapy for 3-6 months, then maintenance to prevent chronic kidney disease. Treatment depends on histologic class and response to therapy. The goal
Systemic lupus erythematosus (SLE) is a multi-system autoimmune disease characterized by autoantibody production and organ damage. It predominantly affects women of childbearing age. The disease has an unknown etiology involving genetic, environmental, and immune system factors. Common clinical manifestations include arthritis, rash, oral ulcers, photosensitivity, and involvement of major organ systems like the kidneys, heart, and lungs. Diagnosis is based on clinical criteria and the presence of autoantibodies. Treatment involves managing symptoms, preventing flares, and suppressing the immune system using medications like corticosteroids, antimalarials, azathioprine, and cyclophosphamide. Pro
AUTOIMMUNITY AND AUTO IMMUNE DISEASES.pdfnithyatulasi1
The immune system could go awry and, instead of reacting against foreign antigens, could focus its attack on self-antigens. Paul Ehrlich termed this condition “horror autotoxicus.”
Mechanisms of self-tolerance normally protect an individual from potentially self-reactive lymphocytes, there are failures. They result in an inappropriate response of the immune system against self-components termed autoimmunity.
AUTOIMMUNITY AND ITS ASSOCIATED DISEASES.pdfnithyatulasi1
The immune system could go awry and, instead of reacting against foreign antigens, could focus its attack on self-antigens. Paul Ehrlich termed this condition “horror autotoxicus.”
Mechanisms of self-tolerance normally protect an individual from potentially self-reactive lymphocytes, there are failures. They result in an inappropriate response of the immune system against self-components termed autoimmunity.
Anaesthesia for neurological and neuromuscular disease2Kanika Rustagi
The document discusses various neurological and neuromuscular diseases relevant to anaesthesia including epilepsy, multiple sclerosis, Guillain-Barre syndrome, poliomyelitis, and cerebral palsy. It covers the pathophysiology, clinical features, diagnostic criteria, and anaesthetic considerations for managing patients with these conditions. Key points discussed include preoperative assessment and planning, choice of anaesthetic agents to avoid exacerbating symptoms, special monitoring needs, and postoperative care considerations.
Agranulocytosis is a condition involving a severe decrease in neutrophils in the blood, increasing the risk of infection. It can be caused by decreased neutrophil production due to conditions like aplastic anemia or cancer treatments, or increased neutrophil destruction from infections, autoimmune disorders, or drug reactions. Symptoms include fever, chills, and mouth sores. Diagnosis is made through blood tests showing very low neutrophil counts. Treatment focuses on managing infections with antibiotics, stimulating new neutrophil production with growth factors, and addressing any underlying causes.
This document provides information about systemic lupus erythematosus (SLE). It defines SLE as a multi-system autoimmune disease affecting various organs mediated by autoantibodies and immune complexes. The causes are unknown but may involve genetic, hormonal, and environmental factors. SLE can affect many organ systems like the kidneys, lungs, heart, and nervous system, causing a variety of clinical manifestations. Diagnosis involves evaluating symptoms, medical history, physical exam, and laboratory tests. Treatment depends on disease severity and organ involvement, and may include medications like NSAIDs, antimalarials, corticosteroids, and cytotoxic drugs. Special considerations are given to SLE in pregnancy and neonates.
Systemic Lupus Erythematosus (SLE) is an autoimmune disease that can affect multiple organ systems. It typically affects young women and is characterized by periods of disease flares and remissions. While the exact cause is unknown, genetic and environmental factors likely contribute to abnormal immune system functioning and production of autoantibodies. Common clinical manifestations include malar rash, arthritis, kidney problems, and hematological abnormalities. Diagnosis involves identifying clinical features and detecting autoantibodies such as antinuclear antibodies and anti-dsDNA antibodies. With proper management, 10-year survival rates are over 90%.
Nephritis is a inflammation of kidney .
It is classified into various types like lupus nephritis ,interstitial nephritis , glomerulonephritis ,pyelonephritis.
Lupus nephritis is an inflammation of kidney due to autoimmune disorder named as lupus .
It is inflammation of lower urinary tract .
1. White blood cells (leukocytes) include granulocytes like neutrophils, eosinophils, and basophils which fight infection, and agranulocytes like monocytes and lymphocytes which are involved in immune responses.
2. Leukemia is a cancer of the blood cells characterized by abnormal proliferation of white blood cells. The four main types are acute myeloid leukemia, acute lymphoblastic leukemia, chronic myeloid leukemia, and chronic lymphocytic leukemia.
3. Factors involved in leukemia development include genetic mutations, chromosomal translocations, radiation exposure, certain chemicals, and some viruses. Maintaining overall health can help support white blood cell counts.
This document provides an overview of systemic lupus erythematosus (SLE). It discusses the definition, epidemiology, pathogenesis, diagnosis, clinical manifestations, management, and complications of SLE. The pathogenesis involves genetic susceptibility and environmental triggers leading to abnormal immune responses and autoantibody production. Diagnosis is based on the SLICC classification criteria. Management involves controlling symptoms, preventing organ damage, and treating flares and complications using medications like glucocorticoids, antimalarials, immunosuppressants, and biologics. Life-threatening complications can include renal disease, neurological involvement, hematological abnormalities and vasculitis.
The document discusses diseases of the pericardium, including acute pericarditis, constrictive pericarditis, pericardial effusion, and cardiac tamponade. It describes the anatomy and functions of the pericardium, symptoms and signs of different pericardial diseases, diagnostic tests including ECG, echo, CT and treatment approaches.
Creative Restart 2024: Mike Martin - Finding a way around “no”Taste
Ideas that are good for business and good for the world that we live in, are what I’m passionate about.
Some ideas take a year to make, some take 8 years. I want to share two projects that best illustrate this and why it is never good to stop at “no”.
A Visual Guide to 1 Samuel | A Tale of Two HeartsSteve Thomason
These slides walk through the story of 1 Samuel. Samuel is the last judge of Israel. The people reject God and want a king. Saul is anointed as the first king, but he is not a good king. David, the shepherd boy is anointed and Saul is envious of him. David shows honor while Saul continues to self destruct.
A Free 200-Page eBook ~ Brain and Mind Exercise.pptxOH TEIK BIN
(A Free eBook comprising 3 Sets of Presentation of a selection of Puzzles, Brain Teasers and Thinking Problems to exercise both the mind and the Right and Left Brain. To help keep the mind and brain fit and healthy. Good for both the young and old alike.
Answers are given for all the puzzles and problems.)
With Metta,
Bro. Oh Teik Bin 🙏🤓🤔🥰
2. DEFINITION
• Systemic Lupus Erythematosus is a chronic Multisystem ,
autoimmune disease In which organs and cells undergo
damage Initially mediated by tissue binding autoantibodies
and immune complexes.
• Females is more common affected than males.
• Common in Reproductive age group
• Prevalence of SLE in united states is 81-144 Per 1 lakh
population.
• Less prevalence in Africa and Australia.
3. PATHOGENESIS OF SLE
• AUTOANTIBODY PRODUCTION:
• B cells of the immune system produce autoantibodies that Target
the body’s own cells and tissues
• Autoantibodies commonly found in SLE is Antinuclear antibodies.
• IMMUNE COMPLEX FORMATION:
• Binding of autoantibodies to self antigens leads to formation of
Immune complexes.
• Immune complexes consist of autoantibodies can circulate in the
blood and deposit in various tissues including kidneys, skin,joints
4. • COMPLEMENT ACTIVATION:
• Immune complexes formed in SLE can activate the classical pathway of
complement system.
• Thus results in release of Inflammatory mediators such as C3a,C5a and the
formation of Membrane attack complex(MAC) ,which can directly damage cells
• INFLAMMATION AND TISSUE DAMAGE:
• Deposition of Immune complexes in tissues and activation of complement
system leads to chronic inflammation due to release of Inflammatory mediators
like cytokines, chemokines.
• Neutrophils, macrophages and other immune cells contribute to tissue damage
through release of enzymes, reactive oxygen species and pro Inflammatory
molecules.
• This chronic inflammation can affect multiple organ systems.
5. • ABNORMAL T CELL FUNCTION:
• In SLE, there is an imbalance of T cell subsets, particularly increase in
Autoreactive CD4 + Tcells.
• Autoreactive T cells can recognise self antigens presented by APC and provide
help to B cells, leading to production of autoantibodies.
• It directly contribute to tissue inflammation by releasing Pro Inflammatory
cytokines like Interleukin-17(IL-17).
• DEFECTIVE CLEARANCE OF APOPTOTIC CELLS:
• In SLE, there is a defect in clearance of apoptotic cells.
• The release of self antigens from these dying cells can trigger an immune
response and contribute to production of autoantibodies.
6. • TYPE 1 INTERFERON PRODUCTION:
• SLE is characterised by increased production of type 1 Interferons, particularly
Interferon-Alpha.
• Type 1 Interferons are cytokines that play a crucial role in the immune response
to viral infections.
• In SLE, there is activation of Plasmacytoid dendritic cells which are specialized
immune cells that produce large amounts of Interferon- Alpha in response to
immune complex formation.
• The increased production of Type 1 Interferons can further stimulate the immune
system , enhance the presentation of self antigens, and promote inflammation.
7. CLINICAL MANIFESTATIONS OF SLE
• MUSCULOSKELETAL :
• Arthralgia and Non erosive arthritis
are most common (>85%).
• It commonly involves PIP and MIP
joints of the hand along with wrist
and knees.
• About 10% of patients, Deformities
result from damage to Periarticular
tissue causing JACCOUD’S
ARTHROPATHY.
8. CUTANEOUS MANIFESTATIONS
• CLASSIC MALAR RASH :
• Acute Manifestation
• Erythematous( Flat or
raised) Facial rash with a
butterfly distribution
across the malar and
nasal prominences and
sparing of the nasolabial
folds
• It is triggered by Sun
exposure.
• Generalized
photosensitivity and
alopecia.
9. • DISCOID LUPUS:
• Chronic
presentation,Benign
variant of lupus in
which only the skin is
involved
• Discoid rash consists
of erythematous,
slightly raised
patches with
adherent keratotic
scaling and follicular
plugging
• The rash is primarily
seen in scalp and
face.
11. HAEMATOLOGICAL MANIFESTATIONS
• Antibodies that Target each of the cellular blood elements are
responsible for haematological changes.
• RBC:Normocytic normochromic anaemia and hemolytic anaemia
• WBC: Leukopenia particularly Lymphopenia..
• PLATELET: Idiopathic /immune thrombocytopenic purpura induced by
Antiplatelet antibodies.
• CLOTTING FACTORS:Impaired clot formation and haemorrhage.
13. CARDIAC MANIFESTATIONS
• Most frequent- PERICARDITIS
• Serious Manifestation-
MYOCARDITIS AND LIBMAN SACK
ENDOCARDITIS
• Due to Accelerated atherosclerosis
and vasculitis – INCREASED RISK
FOR MYOCARDIAL INFARCTION.
14. PULMONARY MANIFESTATIONS
• MOST COMMON PLEURAL LESION
-PLEURISY WITH OR WITHOUT
PLEURAL EFFUSION.
• Pleural effusion – Exudative with low
C3, ANA test positive in pleural fluid.
• LUNG LESION- PNEUMONITIS,
SHRINKING LUNG SYNDROME,
INTERISTITAL INFLAMMATION
LEADING TO FIBROSIS AND INTRA
ALVEOLAR HAEMORRHAGE.
15. • GASTROINTESTINAL MANIFESTATIONS:
• Non specific diffuse Abdominal pain – AUTOIMMUNE PERITONITIS/
INTESTINAL VASCULITIS.
• MESENTRIC VASCULITIS
• OCULAR MANIFESTATIONS:
• COMMON MANIFESTATION- SICCA SYNDROME AND NON SPECIFIC
CONJUNCTIVITIS.
• SERIOUS MANIFESTATION-RETINAL VASCULITIS AND OPTIC
NEURITIS.
16. RENAL MANIFESTATIONS
• Kidney may be involved in 30-50% of SLE Patients.
• Often asymptomatic in most of the lupus patients.
• Characterized by Proteinuria (>500 mg/24 hours) and or cellular (red
cell) casts.
• Urinalysis should be done in any person suspected of having SLE
followed by regular urinalysis and blood pressure monitoring.
17.
18. OTHER MANIFESTATIONS
• KIKUCHI-FUJIMOTO DISEASE- SPLENOMEGALY AND
LYMPHADENOPATHY.
• ANTIPHOSPHOLIPID SYNDROME
• OSTEOPOROSIS
• COMPLEMENT DEFICIENCIES
• NON HODGKIN’S LYMPHOMA
• LUNG AND HEPATOBILIARY CANCERS.
19.
20.
21.
22. LABORATORY INVESTIGATIONS
• AUTOANTIBODIES DETECTION:
• ANTINUCLEAR ANTIBODY: Best screening test and >90% of the patients show
positive test . It is not specific for SLE.
• ANTI-DOUBLE STRANDED DNA- Common in SLE Patients.
• ANTI SMITH ANTIBODIES- Highly specific for SLE Patients.
• RHEUMATOID FACTOR- Positive in 30% of patients
23. • COMPLETE BLOOD COUNT: ANAEMIA, LEUCOPENIA,
LYMPHOPENIA AND THROMBOCYTOPNephritis-.
• ACTIVATED PARTIAL THROMBOPLASTIN TIME:Prolonged in the
presence of Antiphospholipid antibodies
• ERYTHROCYTE SEDIMENTATION RATE- Monitoring the disease
activity.
• URINALYSIS: Active Nephritis- PROTEINURIA, HAEMATURIA AND
CELLULAR OR GRANULAR CASTS.
• COMPLEMENT LEVELS: Low levels of C3 indicate active disease
especially nephritis
• LE CELL: Phagocytic leukocyte (Neutrophil or macrophage) that has
engulfed the denatured nucleus of an injured cell is positive.
24. IMAGING
• CHEST X RAY- To exclude other pathology ,for pulmonary and
cardiac pathology.
• HIGH RESOLUTION CT: To demonstrate Fibrotic lung.
• MRI BRAIN AND SPINAL CORD
• ECHOCARDIOGRAPHY: To diagnose Pericardial and endocardial
involvement
• RENAL BIOPSY:
• INDICATIONS:
• Confirmed proteinuria of >1gm/ 24 hours.
• Proteinuria >0.5gm/day plus hematuria or cellular casts.
25.
26. MANAGEMENT OF NON LIFE THREATENING
DISEASE.
• NSAID’S are useful analgesics/ anti inflammatories , particularly for
Arthralgia/ Arthritis.
• Adverse effects: NSAID’S INDUCED ASEPTIC MENINGITIS,
ELEVATED SERUM TRANSAMINASES, HYPERTENSION AND
RENAL DYSFUNCTION.
• HYDROXYCHLOROQUINE:: Reduces disease symptoms, prolongs
survival, reduces occur of tissue damage including renal damage
• BELIMUMAB AND ANUFROLUMAB- Effective in persistent disease
activity and fatigue despite Standard therapies.
• TOPICAL SUNSCREEN, ANTIMALARIALS, TOPICAL
GLUCOCORTICOIDS AND TACROLIMUS- LUPUS DERMATITIS.
31. DRUG INDUCED LUPUS
• Drug induced lupus is a type of ANA positive Lupus that appears during therapy with
certain Medications and biological agents.
• Drugs like Antiarrythmics (Eg.Procainamide, Diltiazem,disopyramide and
propafenone), Antihypertensive (Eg.Hydralazine), Methyldopa, Angiotensin-
Converting Enzyme inhibitors, beta blockers,Anti thyroid Propylthiouracil,
Antipsychotic – Chlorpromazine,lithium, Anticonvulsants- Carbamazepine and
phenytoin .
• Commonly associated with ANTI HISTONE ANTIBODIES.
• Predominant in whites, less female predilection.
• Rarely involves kidneys and brain.
• Treatment – Resolves over several weeks after discontinuation of offending
medication.
32. PREGNANCY AND LUPUS
• Rate of fetal loss is increased approximately 2-3 fold in women with SLE.
• Fetal demises is higher in mother with high disease activity ,
Antiphospholipid antibodies, Hypertension and/or Active nephritis.
• Active SLE in pregnant women should be controlled with
Hydroxychloroquine and if necessary Prednisone/Prednisolone at the lowest
effective doses for the shortest time require.
• Azathioprine may be added if these treatments do not supress disease
activity.
• Glucocorticoids should be used with caution because of the adverse fetal
effects at high doses.
• Direct oral anticoagulants should avoid in pregnancy.
• In SLE patients with Antiphospholipid antibodies and prior fetal losses ,
treatment with LMWH + Low dose aspirin as significantly increase the
proportion of live births.
33. NEONATAL LUPUS
• Rash and /or Congenital heart block with or without Cardiomyopathy.
• Presence of ANTI –RO
• Cardiac manifestations of Neonatal lupus are life threatening hence
requires vigilant monitoring of fetal heart rate.
• Treatment- HYDROXYCHLOROQUINE treatment of Anti-Ro postive
mother whose prior infant developed Congenital heart block
significantly reduces the chance that subsequent fetuses will develop
heart block.
• DEXAMETHASONE treatment of mother in whom fetal first or second
degree heart block detected in utero sometimes prevents progression
of heart block.
34. LUPUS DERMATITIS
• Exposure to Ultraviolet light.
• Topical GLUCOCORTICOIDS AND ANTIMALARIALS
(EG:HYDROXYCHLORQUINE) are effective in reducing lesion
severity.
• Systemic treatment with RETINOIC ACID is a useful strategy in
patients with inadequate improvement after these interventions on
adverse effects are potentially severe.
• Extensive Pruritic, bullous or ulcerating dermatitides usually improve
Promptly after institution of systemic Glucocorticoids
35. PREVENTION
• Patient receiving >20 mg of Prednisone daily may be protected from
pneumocystis infections with trimethoprim- Sulfamethoxazole or
atovaquone.
• Postmenopausal women can be partially protected from steroid
induced osteoporosis with calcium supplementation, vitamin D and
either bisphosphonates or denisumab.
• Statin therapies reduce all cause deaths in SLE Patients.
36. EXPERIMENTAL THERAPIES
• Depletion of B cells with OBINITUZUMAB
• Inhibition of B cells by blocking more than one receptor for BAFF
(TELACICEPT)
• Elimination of Plasma cells.
• B cells inhibition through inhibition of BTK
• Inhibition of B/T cell second signal coactivation with CTLA-Ig or anti- CD
40L.
• Inhibition of Innate immune activation via TLR7 or TLR7 and TLR9.
• Induction of regulatory T cells with peptides from immunoglobulin or
autoantigens.
• Inhibition of T effector cells through CD6.
• Targeting lymphocyte migration by modulation of S1P1 receptor.
37. POOR PROGNOSTIC FACTORS
• MALE SEX
• OLDER AGE AT PRESENTATION
• BLACK RACE
• HYPERTENSION
• ANTIPHOSPHOLIPID ANTIBODY SYNDROME
• DIFFUSE PROLIFERATIVE GLOMERULONEPHRITIS
38. CAUSES OF DEATH IN SLE
• INFECTIONS AND RENAL FAILURE
• THROMBOEMBOLIC EVENTS
• CARDIOVASCULAR DEATH
