ppt

BGD SUBGROUP B2Surviving An Unfortunate

Cascade

Surviving An Unfortunate Cascade

HISTORYRheumatic Diseases Which Can Present wi

th Arthritis and RashSLEDiagnostic TestsOrgan/System InvolvementNon-lifethreatening & Life Threatening M

anifestationsManagement/TreatmentSimple Do’s and Dont’sCauses of Osteonecrosis

HISTORY


General Data

Name: ABAge: 29 y/o Gender: FemaleOccupation: Bank teller

Presented with fever, hair loss, malaise & joint pain

PE: Alopecia Tender PIP & Malar rash MCP joints


Laboratory Results: AnemiaANA 1:640 elevated anti-dsDNA antibodieslow C3 compliment

negative VDRL & normal urinalysis

Diagnosis: SLE

Medications given: Prednisone 40mg/d with good

response

*Loss of follow-up


One year aftermalaisevisiual complaints difficulty with thought processing

Diagnosis: CNS involvement 2o to SLE

Medications given: Prednisone 60mg/d with good response


Six months later fever lethargy while on Prednisone 10mg/d

Laboratory results:CXR - bilateral mid & lower

lung field infiltratesLung biopsy – Pulmonary hemorrhage

Diagnosis: Pulmonary Hemorrhage

Management: Intubated & maintained on mechanical ventilation

due to progressive respiratory distress

Medication given: Pulse Methylprednisone 1g/d for 3 days


One month after extubation pain on both hips

Laboratory results: Hip Radiograph -evidence of osteonecrosis


Rheumatic Diseases which can present with

Arthritis & Rash(Differential Diagnosis)


1

Many patients with rheumatological disorders either present with a rash or develop a rash in the course of the disease.

The differential diagnosis of rash and

arthritis is wide, but in most cases a diagnosis can be made on the basis of history, clinical examination, and appropriate blood tests.

ABC of Rheumatology: RASHES AND VASCULITISBMJ 1995;310:1128-1132 (29 April) ,R A Watts, D G I Scott


Arthritis and Rash

Systemic Lupus Erythematosus

Dermatomyositis Scleroderma Still’s Disease Rheumatoid Arthritis Eosinophilic Fasciitis Psoriasis Reiter’s Syndrome Bahcet’s Syndrome Stevens-Johnson Syndrome Erythema Nodosum Vasculitis Cryoglobulinemia Rheumatic Fever Juvenile Rheumatoid

Arthritis

Rubella ArthritisGonococcal ArhtritisSubacute Bacterial

EndocardititsUlcerative Colitis &

Regional EnteritisHepatitis-Associated

ArthritisLyme ArthritisArthritis with Pancreatitic

DiseaseLipoid Dematoarthritis

(Multicentric Reticulohistiocytosis)

Arthritis with AcneSweet’s Syndrome


Dermatomyositis


60 y/o woman noticed the appearance of erythematous plaques on the hands, elbows and periorbital areas with weakness, diffuse arthralgia, fatigue, and weight loss.

PE:

•Heliotrope(violaceous) periorbital erythema

•Erythematous plaques (Gottron papules) overlying the knuckles and the elbows

SclerodermaSclerodermaPatient with 20 year history of Raynaud's phenomenon and hand stiffness. She noticed the appearance of several telangectasia on the skin and started suffering from dysphagia and heartburn.

PE:

•Widespread telangectasia involving the face, upper trunk & extremities

•Radial furrows

•“Neck sign” with beading, ridging & hypopigmentation

•Sclerodactyly & pitted scars on the finger-tips

•Mild hypertension

Other Common Rheumatic Disease Presented with Rash & Arthritis:

Rheumatoid Arthritis


•Severe Arthritis & deformation of right & left PIP, MCP, Wrist, elbow, knee, ankle & MTP joints

•Rheumatoid nodule

•Splinter Hemmorhage (Rheumatoid vasculitis)

Psoriatic ArthritisPsoriatic Arthritis

•Asymmetric fusiform swelling of the distal interphalangeal joints

•Round, erythematous plaques with well defined borders covered with white micaceous scales


Bahcet’s Syndrome


Triad:

•Aphthous oral ulcers

•Genital lesions

•Recurrent eye inflammation

_____________

• Arthritis

• Pustular skin lesions

Reiter’s SyndromeReiter’s Syndrome

Triad:

• Urethritis

• Conjunctivitis

• Oligoarthritis

_____________

•Typical rash on the feet


Vasculitis


•Widespread eruption of purpuric macules and papules, ulcers and eschars scattered on the upper andlower extremitis, hands, genitals, face, as well as nasal and oral mucosae.

•Accompanied by severe malaise, fever, arthralgia, paraesthesia, and microhematuria

Still’s DiseaseStill’s Disease

•Fever, joint pain, sore throat, and a rash

•Negative Rheumatoid factor and ANA test


Introduction

ChronicRelapsingInflammatoryMutlisystemic disorder of connective tissueUnknown etiologyAutoimmune dysfunction


Epidemiology

Women>Men (10:1)Reproductive ageMonozygotic twins


““SLE is primarily a disease of women; 85 SLE is primarily a disease of women; 85 percent of sufferers are female. Its percent of sufferers are female. Its onset is usually between 12 to 50 years onset is usually between 12 to 50 years old. SLE has a racial predisposition, old. SLE has a racial predisposition, being four times more common in being four times more common in blacks than in whites.”blacks than in whites.”

http://www.bayanihan.org/html/public_html/article.php?http://www.bayanihan.org/html/public_html/article.php?story=20030914230114038story=20030914230114038

Epidemiology


Clemente M. Amante, MD, UPPGH, “Clinical Review of SLE”, Medical Progress, April 1999

Criteria for Classification of SLEDDiscoid rash iscoid rash Erythematous circular raised patches with Erythematous circular raised patches with

adherent keratotic scaling & follicular plugging; adherent keratotic scaling & follicular plugging; atrophic scarringatrophic scarring

OOral Ulcers ral Ulcers Includes oral & nasopharyngeal ulcers, Includes oral & nasopharyngeal ulcers, observed by phisicianobserved by phisician

PPhotosensitivity hotosensitivity Exposure to ultraviolet light causes rashExposure to ultraviolet light causes rash

AArthritisrthritis Nonerosive arthritis of 2 or more peripheral Nonerosive arthritis of 2 or more peripheral jts, with tenderness, swelling or effusionjts, with tenderness, swelling or effusion

MMalar rashalar rash Fixed erythema, flat or raised, over the Fixed erythema, flat or raised, over the malar eminencesmalar eminences

IImmunolodic disordermmunolodic disorder Anti-dsDNA, anti-Sm, and Anti-dsDNA, anti-Sm, and or/antiphospholipidor/antiphospholipid

NNeurologic disordereurologic disorder Seizures or psychosis without other causesSeizures or psychosis without other causes

RRenal disorderenal disorder Proteinuria.0.5 g/d or ≥3+,or cellular castsProteinuria.0.5 g/d or ≥3+,or cellular casts

AAntinuclear ntinuclear antibodies antibodies

Abnormal titer of ANA by immunofluoroscence or an Abnormal titer of ANA by immunofluoroscence or an equivalent assay at any point in time in the absence of drugs equivalent assay at any point in time in the absence of drugs known to induce ANAsknown to induce ANAs

SSerositiserositis Pleuritis or pericarditis documented by ECG Pleuritis or pericarditis documented by ECG or rub or evidence of effusionor rub or evidence of effusion

HHematologic disorderematologic disorder Hemolytic anemia or leukopenia<4000/uL or Hemolytic anemia or leukopenia<4000/uL or lymphopenia <1500/uL in the absence of offending drugslymphopenia <1500/uL in the absence of offending drugs


Frequency of Lupus Manifestations

ManifestatioManifestationsns

At onsetAt onset AnytimeAnytime

ArthralgiaArthralgia 77%77% 85%85%

ConstitutionConstitutionalal

73%73% 84%84%

SkinSkin 57%57% 81%81%

ArthritisArthritis 56%56% 63%63%

RenalRenal 44%44% 77%77%


John H. Klippen & Paul A. Dieppe, Practical Rheumatology

Diagnostic tests for SLE

Surviving An Unfortunate Surviving An Unfortunate CascadeCascade

•Test for Autoantibodies (Immunological test)

•Standard Screening Test

3

Introduction

In previously undiagnosed patients thought to have SLE, the principal diagnostic study is the ANA test.

A positive ANA alone is not sufficient for diagnosis. Positive test results are seen in other autoimmune conditions and in a certain percentage of the general population


ANA TestImmunofluorescence

technique, which detects patient serum antibodies that react with cell nuclei components, is used. Most sensitive and most commonly used substrate is human HEp-2 epithelial tumor.

ANA detects >95 percent of patients with SLE and drug-induced lupus


Homogenous

Anti-Histones

Speckled

Anti-Sm

Centromere

Anti-Centromere

Nucleolar

Anti-RNA

ANA Test1:40 or 1:80 titers using

HEp-2 substrate considered positive (Px= 1:40)

When a positive ANA result is thought to be clinically relevant, assessment of the presence of antibody level to native, double-stranded DNA (dsDNA antibody) confirm the diagnosis of SLE

SLE activeSLE inactiveDrug-induced lupusMixed connective tissue diseaseSclerodemaSjogren's syndromeMyositis (polymyositis and dermatomyositis)Rheumatoid arthritisJuvenile rheumatoid arthritisDiscoid LEChronic autoimmune hepatitisHashimoto's thyroiditisNormal women

Titer>1:640

1:40

%

999599999575605070304040

4


Disease Commonly Associated a with Positive ANA test

Peter Barland, M.D. The Antinuclear Antibody Test

Typical ANA patterns seen with Indirect Immunofluorescent staining of HEP 2 cells

•Homogeneous (Anti-histone antibodies) - found in patients with drug-induced ANA's and with anti-histone dsDNA complexes found in patients with SLE and rheumatoid arthritis

•Speckled (Anti- RNP antibodies) - found in patients with mixed connective disease, with anti-SM antibodies found in patients with SLE and with other less well-characterized anti-nuclear antibodies found in scleroderma and polymyositis

•Centromere (Anti- centromere antibodies) - found specifically in patients with CREST syndrome

•Nucleolar (Antibodies to ribonuclear proteins and RNA polymerases) - found primarily in patients with scleroderma.

Tan EM. Antinuclear antibodies: Diagnosticmarkers for autoimmune diseases and probes for cell biology. Adv Immunol 1989;44:93-151

Anti-dsDNA> 50 percent SLE patients have antibody that reacts at high titer with native double-stranded DNA. Patients with quiescent disease may test negatively.

Anti-dsDNA antibodies not found in drug-induced lupus.

95 percent specificity anti-dsDNA when test properly conducted

Px= elevetaed anti-dsDNA


Anti-Sm antibodyAnti-Sm antibody Antibody to a Sm antigen designated snRNP Antibody to a Sm antigen designated snRNP

or small nuclear ribonucleoprotein or small nuclear ribonucleoprotein This is seen in approximately one third of This is seen in approximately one third of

SLE patients and is fairly specific for lupus SLE patients and is fairly specific for lupus

Other Autoantibodies & it’s Clinical Association

AntibodAntibodyy

%SLE%SLE Antigen Antigen RecognizedRecognized

Clinical UtilityClinical Utility

Antinuclear Antinuclear AntibodyAntibody

9898 Multiple NuclearMultiple Nuclear Best screening test; repeated negative tests make Best screening test; repeated negative tests make SLE unlikelySLE unlikely

Anti-dsDNAAnti-dsDNA 7070 DNA (double DNA (double stranded)stranded)

High titers are SLE-specific & in some patients High titers are SLE-specific & in some patients correlate with disease activity, nephritis & correlate with disease activity, nephritis & vasculitisvasculitis

Anti-SmAnti-Sm 2525 Protein Complexed Protein Complexed to 6 sp. of nU1 RNAto 6 sp. of nU1 RNA

Specific for SLE; no definite clinical correlationSpecific for SLE; no definite clinical correlation

Anti-RNPAnti-RNP 4040 Protein Protein complexed to U1 complexed to U1 RNAyRNAy

Not specific for SLE; high titers associated with Not specific for SLE; high titers associated with syndrome that have overlap features of several syndrome that have overlap features of several rheumatic syndromerheumatic syndrome

Anti-Ro (SS-A)Anti-Ro (SS-A) 3030 Protein Protein complexed to hY complexed to hY RNARNA

Not specific for SLE; associated with sicca Not specific for SLE; associated with sicca syndrome, subacute cutaneous lupus, & neonatal syndrome, subacute cutaneous lupus, & neonatal lupus w/ congenital heart blocklupus w/ congenital heart block

Anti-La (SS-B)Anti-La (SS-B) 1010 47-kDa Protein 47-kDa Protein complexed to hY RNAcomplexed to hY RNA

Usually associated with Anti-Ro; associated with Usually associated with Anti-Ro; associated with decrease risk of nephritisdecrease risk of nephritis

AntihistoneAntihistone 7070 Histones ass. w/ DNAHistones ass. w/ DNA More frequent in drug-inducaed lupusMore frequent in drug-inducaed lupus

AntiphospholipAntiphospholipidid

5050 PhospholipidsPhospholipids Predispose to clotting, fetal loss & Predispose to clotting, fetal loss & thrombocytopeniathrombocytopenia

AntierythrocytAntierythrocytee

6060 Erythrocyte membraneErythrocyte membrane Develops overt hemolysisDevelops overt hemolysis

AntiplateletAntiplatelet 3030 Platelets surface & Platelets surface & cytoplamcytoplam

Associated with thrombocytopenisAssociated with thrombocytopenis

AntineuronalAntineuronal 6060 Neuronal & Neuronal & lyphocyte surface lyphocyte surface

Correlate with active CNS lupusCorrelate with active CNS lupus

Antiribosomal Antiribosomal PP

2020 Proteins in Proteins in RibosomesRibosomes

Correlates with depression or psychosis due to Correlates with depression or psychosis due to CNS lupusCNS lupus

4

Additional Immunological TestsComplement studies (C3, C4, CH50) may

be useful to determine disease activity in patients known or thought to have SLE

A decreased C3 (<64 mg/dL) and C4 (<15 mg/dL) level are often associated with active disease in SLE patients (35-60%)

Px= low C3 complement


Agnello V. Association of systemic lupus erythematosus and systemic lupus erythematosus-like syndromes with hereditary and acquired complement deficient states. Arthritis Rheum

1978;21:S146

Standard Test for Diagnosis

Complete Blood CountPlatelet CountUrinalysis

ORGAN/SYSTEM INVOLVEMENT


2

Cognitive dysfunction particularly difficulty with memory and reasoning

Headaches

- indicates SLE flare when excruciating

- difficult to distinguish from migraine or tension

headaches when milder

Seizures

Nervous System Manifestations


Bluestein, HG. The neuropsychiatric manifestations of SLE. In: Weisman MM, Weinblatt ME, eds. Treatment of the Rheumatic Diseases. Philadelphia: W.B. Saunders;

1995:128-136.

Nervous System Manifestations “Neurologic manifestation in a Filipino group of patient seen at UST Hospital from 1981 to 1993 showed that fifty-five out of 170 (32%) SLE patients manifested with neurologic problems during the course of their illness.”

“The occurrence of neurologic manifestation in systemic lupus erythematosus has varied from 25 to 60%... It has generally been apparent that seizures, psychosis, cranial nerve signs & cerebrovascular accidents are the leading presentations in SLE.”

Ma Carmencit Gonzalez, MD, Sandra Teres G. Victorio-Navarra, MD, FPCP, FPRA, Jesus F. Poblete, MD, FPNA & Tito P. Torralba, M.D., FPCP, FPRA. Neurologic Manifestation of Systemic Lupus Erythematosus among Filipinos. Phil. J. Internal Medicine, 32:23-27, Sept-Oct 1994

Nervous System Manifestations

“SLE frequently involves the central nervous system & this involvement often develops terminally… Convulsive disorders and disturbance of mental function are the most common neurologic sign in SLE. The prevalence of microinfarcts in the cerebral cortex probably accounts for the predisponderance of these signs.” “The effect of steroid treatment on the neurological manifestation is uncertain… Seizures and mental disorder may be precipitated or accentuated by steroids, while in other cases, steroids bring about improvement in these disorders… Treatment by steroids must be determined by the response observed in the individual patient.”

Rosella P. Autor, MD, Ma Carmencita B. Gonzales, MD & Artemio T. Ordinario, MD, FPNA. “Neuropathologic findings in systemic lupus erythematosus: a report in 4 cases. Santo Tomas Journal of Medicine, Vol 42 No. 6 Nov-Dec 1993

Musculoskeletal Manifestations

Myalgias and arthralgias are present most of the time in the course of the illness

Mild to disabling intermittent polyarthritis characterized by soft tissue swelling and tenderness (commonly in hand, wrists and knee joints)

Active systemic disease suggested by presence of visible synovitis

Increased prevalence of ischemic necrosis of bone

Myositis with muscle weakness, necrosis and inflammation


Cronin ME. Musculoskeletal manifestations of systemic lupus erythematosus. Rheum Dis Clin North Am 1988;14:99-116.

Malar rash

- most common SLE rash

- photosensitive, slightly raised erythema, occasionally scaly on the face particularly the cheeks and nose (butterfly rash), ears, chin, V region of the neck, upper back and extensor surfaces of the arms.

Discoid Lupus Erythematosus (DLE) - 20%

Cutaneous Manifestations


- roughly circular discoid lesions with slightly raised, scaly, hyperpigmented erythematous rims and depigmented atrophic centers in which all dermal appendages are permanently destroyed.

Cutaneous Manifestations

Subacute Cutaneous Lupus Erythematosus (SCLE)

- scaly red patches similar to psoriasis or attacks of circular red-rimmed lesions

Oral ulcers

- lesions resemble aphthous ulcers, indicates systemic disease activity

Alopecia


Laman SD, Provost TT. Cutaneous manifestations of lupus erythematosus. Rheum Dis Clin North Am 1994;20(1):195-212

Ocular Manifestations

Sicca syndromeNonspecific conjunctivitis

- common in SLE and rarely threatens vision

Retinal vasculitis and optic neuritis- serious manifestations- blindness can develop over days to

weeks


Pulmonary hemorrhage

- Due to pulmonary vasculitis

Pleuritis with or without pleural effusion

Pulmonary infiltrates

- manifestation of active SLE, difficult to distinguish from infection

Interstitial inflammation leading to fibrosis

Intraalveolar hemorrhage

Pulmonary Manifestations


Pulmonary Manifestations “Cardiopulmonary manifestations occur in 40-50% of patient with SLE… The clinical spectrum ranges from mild, self-limited, pleuritic chest pain to fulminant & rapidly fatal, difuse, pulmonary hemorrhage… Immune complex deposition plays a significant role in the pathogenesis of these pulmonary manifestation.” “No pulmonary process should be attributed to lupus until infection has been rigorously excluded in SLE patients.” Marian B. Buenviaje, MD, FPCP, Sandra Teres G. Victorio-Navarra, MD, FPCP, FPRA, & Tito P. Torralba, M.D., FPCP, FPRA. Pulmonary Manifestation of Systemic Lupus Erythematosus among Filipinos. Phil. J. Internal Medicine, 32:18-22, Sept-Oct 1994

Pulmonary Pulmonary ManifestationsManifestations

“SLE-related pulmonary hemorrhage requires heroic intervention because of a 60% mortality rate. Treatment with pulse methylprednisilone 1000 mg/d along with cyclophosphamide, ventilator assistance in an ICU setting, intravenous immunoglobulin, and apheresis are among the modalities that have been tried. “Interstitial lung disease either from the SLE or the drugs used in the treatment of lupus occurs occasionally. An open lung biopsy may be needed to differentiate between an infectious agent and an autoimmune mediated process. The acute process is treated with high doses of corticosteroids often followed by cytotoxic drugs used in other high-risk manifestations of lupus.”

Schwab EP, Schumacher Jr HR, Freundlich B, et al. Pulmonary alveolar hemorrhage in systemic lupus erythematosus. Semin Arthritis Rheum 1993;23:8-15.

Nephritis

- Diffuse Proliferative Glomerulonephritis (DPGN)

- the most serious manifestation of SLE

- asymptomatic in most patients

- urinalysis reveals microscopic hematuria and proteinuria (>500mg/24 hr)

- if untreated, patients develop End-Stage Renal

Disease (ESRD) within 2 years of diagnosis

Renal Manifestations


Gladman DD, Urowitz MB, Cole E, et al. Kidney biopsy in SLE. I. A clinical-morphologic evaluation. Quart J Med 1989;73:1125-1153.

Pericarditis

- most frequent, infrequently leads to tamponade

- responds to anti-inflammatory therapy

Myocarditis

Fibrinous endocarditis of Libman-Sachs

- endocardial involvement can lead to valvular insufficiencies (mitral or aortic valves) or to embolic events

Myocardial infarction

- due to accelerated atherosclerosis

Cardiac Manifestations


Cardiac Manifestations

“Cardiovascular involvement including hypertension in SLE has been reported in 38-89% of cases… It involve the pericardium, myocardium, endocardium, coronary arteries conduction of tissues & the blood vessels. This may result from immune complex deposition in the involved tissues, co-morbid illnesses associated with the disease itself such as hypertension, anemia, fever, CHF, renal & pulmonay disease, or they may be the effects of treatment regimens.”Miriam B. Buenviaje, MD, FPCP, Sandra teresa G. Victorio-Navarra, MD, FPCP & Tito P. Torralba, MD, FPCP, “Cardiovascular Manifestation of Systemic Lupus Erythematosus – Santo Tomas University Hospital Experience. Phil. J. Internal Medicine, 32:13-17, Sept-Oct 1994

Hematologic ManifestaionsAnemia

- usually normochromic normocytic

- most frequent hematologic manifestation reflecting chronic illness

LeucopeniaThrombocytopeni

*if platelet counts are >40,000/uL and abnormal bleeding is absent, therapy may not be required


Clemente M. Amante, MD, UPPGH, “Clinical Review of SLE”, Medical Progress, April 1999

Gastrointestinal Manifestations

Nonspecific - nausea, vomiting, diarrhea, diffuse

abdominal pain

Abnormal liver enzymes- serum AST and ALT are increased- common when SLE is active

Vasculitis involving the intestine- life-threatening- complications are perforation,

ischemia, bleeding and sepsis


Hoffman BI, Katz WA. The gastrointestinal manifestations of systemic lupus erythematosus: a review of the literature. Semin Arthritis Rheum 1980;9:237-247

SYSTEMIC LUPUS ERYTHEMATOSUS DISEASE ACTIVITY INDEXSELENA MODIFICATIONPhysicians Global Assessment0 None 1 Mild 2 Med 3 Severe

WtWt DescriptorDescriptor DefinitionDefinition

88 Seizure Seizure Recent onset. Exclude metabolic, infectious or drug causeRecent onset. Exclude metabolic, infectious or drug cause

88 Psychosis Psychosis Altered ability to function in normal activity due to severe Altered ability to function in normal activity due to severe disturbance indisturbance in

the perception of reality. Include hallucinations, incoherence, the perception of reality. Include hallucinations, incoherence, markedmarked

loose associations, impoverished thought content, marked illogicalloose associations, impoverished thought content, marked illogical

thinking, bizarre, disorganized, or catatonic behavior. Excluded thinking, bizarre, disorganized, or catatonic behavior. Excluded uremiauremia

and drug causes.and drug causes.

88 Organic BrainOrganic Brain

SyndromeSyndromeAltered mental function with impaired orientation, memory or otherAltered mental function with impaired orientation, memory or other

intelligent function, with rapid onset fluctuating clinical features. intelligent function, with rapid onset fluctuating clinical features. IncludeInclude

clouding of consciousness with reduced capacity to focus, and clouding of consciousness with reduced capacity to focus, and inability toinability to

sustain attention to environment, plus at least two of the following:sustain attention to environment, plus at least two of the following:

perceptual disturbance, incoherent speech, insomnia or daytimeperceptual disturbance, incoherent speech, insomnia or daytime

drowsiness, or increased or decreased psychomotor activity. drowsiness, or increased or decreased psychomotor activity. ExcludeExclude

metabolic, infectious or drug causes.metabolic, infectious or drug causes.


88 VisualVisual Retinal changes of SLE. Include cytoid bodies, retinal hemorrhages, Retinal changes of SLE. Include cytoid bodies, retinal hemorrhages, serious exodate or hemorrhages in the choroids, or optic neuritis. serious exodate or hemorrhages in the choroids, or optic neuritis. Exclude hypertension, infection, or drug causes.Exclude hypertension, infection, or drug causes.

88 Cranial NerveCranial Nerve New onset of sensory or motor neuropathy involving cranial New onset of sensory or motor neuropathy involving cranial nerves.nerves.

88 Lupus Lupus Headache Headache

Severe persistent headache: may be migrainous, but must be Severe persistent headache: may be migrainous, but must be nonresponsive to narcotic analgesia.nonresponsive to narcotic analgesia.

88 CVA CVA New onset of cerebrovascular accident(s). Exclude New onset of cerebrovascular accident(s). Exclude arteriosclerosis arteriosclerosis

88 Vasculitis Vasculitis Vasculitis Ulceration, gangrene, tender finger nodules, periungual, Vasculitis Ulceration, gangrene, tender finger nodules, periungual, infarction, splinter hemorrhages, or biopsy or angiogram proof of infarction, splinter hemorrhages, or biopsy or angiogram proof of vasculitis.vasculitis.

44 Arthritis Arthritis More than 2 joints with pain and signs of inflammation (i.e. More than 2 joints with pain and signs of inflammation (i.e. tenderness, swelling, or effusion).tenderness, swelling, or effusion).

44 Myositis Myositis phosphokinase/adolase or electromyogram changes or a biopsy phosphokinase/adolase or electromyogram changes or a biopsy showing myositis.showing myositis.

44 Urinary Urinary Casts Casts

Heme-granular or red blood cell casts Heme-granular or red blood cell casts

44 Hematuria Hematuria >5 red blood cells/high power field. Exclude stone, infection or other >5 red blood cells/high power field. Exclude stone, infection or other cause.cause.


44 Proteinuria Proteinuria >0.5 gm/24 hours. New onset or recent increase of more >0.5 gm/24 hours. New onset or recent increase of more than 0.5 gm/24 hours.than 0.5 gm/24 hours.

44 Pyuria Pyuria >5 white blood cells/high power field. Exclude infection.>5 white blood cells/high power field. Exclude infection.

22 New Rash New Rash New onset or recurrence of inflammatory type rash.New onset or recurrence of inflammatory type rash.

22 Alopecia Alopecia New onset or recurrence of abnormal, patchy or diffuse loss New onset or recurrence of abnormal, patchy or diffuse loss of hair.of hair.

22 Mucosal Mucosal Ulcers Ulcers

New onset or recurrence of oral or nasal ulcerations New onset or recurrence of oral or nasal ulcerations

22PleurisyPleurisy Pleuritic chest pain with pleural rub or effusion, or pleural Pleuritic chest pain with pleural rub or effusion, or pleural

thickening.thickening.

22PericarditisPericarditis Pericardial pain with at least 1 of the following: rub, effusion, or Pericardial pain with at least 1 of the following: rub, effusion, or

electrocardiogram confirmation.electrocardiogram confirmation.

22 Low Low Complement Complement

Decrease in CH50, C3, or C4 below the lower limit of normal Decrease in CH50, C3, or C4 below the lower limit of normal for testing laboratory.for testing laboratory.

22 Increased Increased DNA bindingDNA binding

>25% binding by Farr assay or above normal range for >25% binding by Farr assay or above normal range for testing laboratory.testing laboratory.


Fever Fever >38°C. Exclude infectious cause>38°C. Exclude infectious cause

ThrombocytThrombocyto-penia o-penia

<100,000 platelets/mm3 <100,000 platelets/mm3

Leukopenia Leukopenia <3,000 White blood cell/mm3. Exclude drug causes.<3,000 White blood cell/mm3. Exclude drug causes.

Patient’s SLE-DAI Score

Upon ConsultationUpon Consultation Six Months LaterSix Months Later After 1 YearAfter 1 Year

Fever 1Fever 1

Alopecia 2Alopecia 2

Malaise 4Malaise 4

Arthritis 4Arthritis 4

Malar rash 2Malar rash 2

Low Complement Low Complement 22

Increase DNAIncrease DNA

Binding 2Binding 2

----------

Total 17Total 17

Fever 1Fever 1

Lethargy 8Lethargy 8

--------------

Total 9Total 9

After 1 YearAfter 1 Year

Malaise 4Malaise 4

Visual 8Visual 8

Chronic BrainChronic Brain

Syndrome 8Syndrome 8

----------

Total Total 2020

Non-Life Threatening and Life-Threatening SLE

Manifestations


5

Non-Life Threatening SLE Manifestation

Musculoskeletal Manifestations Arthralgias and Myalgias Non-erosive Polyarthritis Hand deformities

Cutaneous Manifestations Photosensitivity Malar rash Oral ulcers Alopecia


Non-Life Threatening SLE Manifestation

Neurologic Headache

Ocular

Retinal Vasculitis

Conjunctivitis


Life-threatening SLE Manifestations

Neurologic

Aseptic Meningitis

Seizures

Subarachnoid HemorrhageRenal

Glomerulonephritis

Renal Failure



Cardiopulmonary System

Pericarditis

Myocarditis

Valvular insufficiency

MI

Pleural effusions

Pulmonary hypertension

ARDS/Pulmonary hemorrhage



Gastrointestinal

Diarrhea

Intestinal Vasculitis and intestinal

perforation

Acute pancreatitisThrombosis


Association of Infections with SLE

“Infections remain an important cause of morbidity & mortality of Systemic lupus erythematosus… In a study of 160 Infections in 122 SLE patients, the most common infections were tuberculosis (29%), pneumonia (21%), Herpes zoster (15%), soft tissue abcess (11%) & septic arthritis (5%).” “Patients with terminal infections had a shorter disease duration, low WBC count & serum C3 levels. This subset of patients also have higher prednisone doses (>30mg/d) in addition to immunologic agents, factors likely contributory to a poorer outcome & high mortality rate.”Caroline G. Arroyo, MD, Sandra teresa G. Victorio-Navarra, MD, FPCP & Tito P. Torralba, MD, FPCP, “Infections among filipinos with systemic lupus erythematosus”. Phil. J. Internal Medicine, 32:45-50, Sept-Oct 1994

Management / Treatment


6

Treatment AlgorithmLife/organ threatening disease

Yes No

Requiring immunosuppression

Yes No

High dose GC

Response and toxicityAcceptable?Taper GC to lo maintenance Dose.

Response inadequateToxicity intolerable•Add cytotoxic •Add immunossupressive

Alternative theraphy•Anticoagulant•Splenectomy•Psychoactive drugs

Response/toxicity acceptable•Slowly taper GC and Cytotoxic drugs•Maintenance Preps•Discontinue if possible

Response/toxicity unacceptable•Add experimental theraphy

•Mycophenolate•Cyclosporine•Plasmapheresis•combinations

Conservative measures•Analgesics•Local steroids •Sunscreens•NSAIDS•Salicylate•Antimalarials•DHEA

Quality of life

AcceptableNo Change

Not acceptableUse low dose GC


Management Goals

Individualized conforming to patient's manifestations.

Reversal of the immune dysregulation and inflammation

Prevention of permanent organ dysfunctionImprovement of symptoms


Drug Regimens

NSAIDSCorticosteroidsAnti-malarialsImmunosuppressives


NSAIDS

Provide symptomatic relief for arthralgias, fever, and mild serositis

NSAIDs may cause elevated liver function tests in patients with active SLE

Ibuprofen is DOC with patients with mild to moderate pain

Also inhibits inflammation by inhibition of prostaglandin synthesis


NSAIDS

400 mg PO q4-6h, 600 mg q6h, or 800 mg q8h while symptoms persist; not exceeding 2.4 g/d

Contraindications:HypersensitivityPeptic ulcer diseaseRecent GI bleeding Renal insufficiency


Corticosteroids

Decrease inflammation and immunosuppressantDecrease inflammation by reversing

increased capillary permeability Suppressing PMN activityStabilizes lysosomal membranes Suppresses lymphocytes and antibody

production


Two Types of Therapy

Prednisone - 5-60 mg/d PO qd or divided bid/qid; taper over 2 wk as symptoms resolveThis regimen is used for milder SLE

symptoms

Methylprednisolone - 1 g/d IV for 3 dUsed for acute organ-threatening

exacerbations.


Contraindications for Corticosteroid Use

Hypersensitivityviral infectionpeptic ulcer diseasehepatic dysfunctionconnective tissue infectionsfungal tuberculosis GI disease


Precautions for Usage

hyperglycemia edema osteonecrosis myopathy peptic ulcer

disease hypokalemia

- osteoporosis- euphoria- psychosis- myasthenia gravis- growth

suppression- infection

Abrupt discontinuation of Abrupt discontinuation of glucocorticoids may cause adrenal glucocorticoids may cause adrenal crisis:crisis:


Complications

Opportunistic infections: TB VZV Herpes Fungal

Osteonecrosis, especially of the hips and knees, is not uncommon and is related to prolonged high-dose corticosteroid usage.

Premature atherosclerotic disease and myocardial infarction are possible complications


Prevention

It is therefore prudent to make sure that the patient is cleared of any infection (especially of TB, viral, and fungal) before giving a corticosteroid treatment regimen.


Other Modes of Treatment

Immunossuppressives • Act as immunosuppressives, cytotoxic and

anti-inflammatory

Methotrexate - 7.5-15 mg PO/IM qwkCyclophosphamide - 500-750 mg/m2 IV every

mo

Hydroxychloroquine - 200 mg PO qd/bid, depending on response


Simple Do’s and Dont’s in Patients with SLE


7

DO’s for the patient

Do learn what your drugs are and what they are for. Take as prescribed, Call side effects to your doctor's attention. Be aware of the drugs that can irritate Lupus, and to which the patient is allergic

Check your doctor before having surgery or dental work performed

Avoid contact with contagious or infectious diseases

Contact your doctor when infections, injuries, or signs of flare ups occur

Do allow yourself recuperation time after hospital stays, flare ups, surgeries, or infections



Do avoid prolonged exposure to direct sunlight and other ultra violet rays, especially if you are one of those extra sensitive patients.

Use sunscreen lotions with a high protective factor, hats, long sleeves, etc

Remember that ultra violet rays bounce off cement, snow, water, and black disco lights, and that being in the shade may not always protect you

Avoid tanning salons and sun lamps

Do eat well balanced meals. Use special diets or vitamins when they are prescribed for a special need. Avoid salt and salty foods while on steroids or when kidney problems exist.



Do learn as much about your disease as you can, and learn to recognize some of the warning signs of disease activity. Remember that SLE differs in each patient, and that you may never develop some of the symptoms observed in other patients.

Do avoid physical and mental stress

whenever posible, yet keep as active as you can within your limits. Include rest periods in your schedule.

Do remember, there is every reason to hope for a brighter future. Researchers say we are near breakthroughs and exciting research is being done.


DONT’s for the patient

Don't experiment with your drugs, take drugs prescribed for others, take a drug you are not familiar with, take an over the counter drug your doctor has not approved for you, stop steroids abruptly.

Don't give up on your treatment until you have given it a fair chance and have followed your doctor's advice, Don't fall for quack treatment.

Don't resume normal activities too fast after your body has been under stress.



Don't hibernate from the sun initially, as long as you are protected and do not stay too long. Some patients can tolerate more than others.

Don't use fad diets. Don't go on a weigh reduction program without your doctor's approval

Don't be ashamed of your disease or symptoms. Don't talk all the time about your problems. Don't let lupus come your life, but accept it as part of your life. Don't forget that your family is under stress too, when you're ill



Don't feel you are abnormal because you have periods of feeling helpless or depressed. Don't hesitate to seek advice if such feelings continue.

Don't ever feel that your case is hopeless. The treatment of Lupus is becoming more effective and the disease can be controlled reasonably well in most people.

Don't say there is nothing you can do to help yourself or others.


ppt

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