SYSTEMIC LUPUS ERYTHEMATOSUS REVIEW OF SYSTEMS

(Dra. Saguil-Sy online class, 2021)

 General: (+) fever, (+) weight loss, (+) anorexia, (+)
generalized body weakness, (+) morning fatigue, (-) easy
Case bruisabillty
GENERAL DATA:  Skin and joints: (+) itchiness, (+) rash, (-) pallor, (-) jaundice, (-
 KC ) hyper/hypopigmentation, (-) photosensitivity, (-) joint
 30 year old female stiffness, (-) joint pain, (-) cramps
 VMMC employee  Head and Neck: (+) Headache,(+) dizziness, (-) syncope, (-)
 Married seizures, (-) blurred vision, (-) redness, (J itchiness, (-)
 Roman Catholic excessive lacrimation,(-) tinnitus, (-) aural discharge, (-) nasal
 Quezon City discharge, (-) gum bleeding, (sore throat
 Respiratory: (+) non-productive cough, (-) dyspnea, (-)
CHIEF COMPLAINT hemoptysis, (-) pain associated with respiration or movement
 Dry, cracked bleeding lips
 Cardiovascular: (-) chest pain, (-) palpitations, (-) easy
HPI fatigability, (-) shortness of breath
 Gastrointestinal: (-) diarrhea, (-) constipation, (-) dysphagia, (-
) hematemesis, (-)vomiting
 Genitourinary: (-) hematuria, (-) bubbly urine, (-) nocturia, (-)
bipedal edema, (-) tremors
PHYSICAL EXAMINATION
 Conscious, coherent, ambulatory with
assistance, not in cardiorespiratory
distress, BMI 19 BP 110/70mmHg, CR
85bpm, RR
 20cpm, T 38.1 C, 02 sat 96%
 (+) flushed skin
 (+) butterly-shaped rash over the malar area and the bridge
of the nose but sparing the nasolabial fold
 (+) erythematous annular esions with raised scaly borders
over the face, arms and lower extremities
 Pink palpebral conjunctivae, anicteric sclerae, no nasoasural
discharge, (+) dry cracked lips with crusted blood, moist
buccal mucosa, with "curdled milk" like material on the hard
palate and tongue that bleeds when removed
 Supple neck, no vein engorgement, no palpable cervical
lymph node, no palpable mass, thyroid not enlarged
FAMILY HISTORY Symmetrical chest expansion, no retractions, (+) fine crackles
 (+) Renal cell carcinoma — father on both bases, no wheezing
 (-) Hypertension  Adynamic precordium, normal rate, regular rhythm, no
 (-) Diabetes Mellitus appreciable murmur
 (-) Pulmonary tuberculosis  Flat abdomen, soft, non-tender, no masses palpated
 (-) Heart disease  Grossly female external genitalia, no ulcers, no masses
 (-) Malignancy Pulses full and equal, no cyanosis, no edema, no joint
 (-) Connective tissue diseases swelling
PERSONAL AND SOCIAL HISTORY NEUROLOGICALEXAMINATION
 Non-smoker  GCS 15 (E4V5M6), Oriented to Time, Place and Person
 Non-alcoholic beverage drinker  Cranial Nerves
 Denies illicit drug use  Olfactory: Intact optic: Pupils 2-3mm ERTL
 Mixed diet with some preference for meat and vegetables  Occulomotor, Trochlear and Abducens: Extraocular Muscles
 Works as a pharmacist in VMMC, primarily involved in Intact with full and Equal Movement
dispensing medicines. Does not come in contact with raw  Trigeminal: Can raise eyebrows, puff cheeks, smile
materials or other chemicals.  Facial: No noted facial asymmetry
OB-GYN HISTORY  Vestibulocochlear- No noted hearing deficits
 G2P2 (2002)  Glossopharyngeal and Vagus: Intact Gag Reflex, Uvula
Midline
 I-MP: January 28, 2014 (Interval: 30 days, Duration: 5 days,
Amount: 2-3 fully soaked pads per day, No dysmenorrhea)  Accessory: Can Turn head side to side against resistance, can
shrug both shoulders equally
 One sexual partner — Husband
 Hypoglossal: Tongue Midline on Protrusion
 No dyspareunia or post-coital bleeding
 No history of sexually transmitted diseases  MMT 5/5 all Extremities
 No Babinski
 No history of recurrent abortions
 No Nuchal Rigidity
 No history of preeclampsia or eclampsia
 No known gynecologic problem  No Noted Sensory Deficits
INITIAL IMPRESSON
 Psoriasis vs. Tinea Corporis
 Aphthous ulcers, buccal area

LABORATORIES

SALIENT FEATURES
 Butterfly-shaped rash on malar area and bridge of the nose
but sparing the nasolabial fold
 Annular erythematous lesions with raised scaly borders over
the face, arms and lower extremities Dry cracked lips with
crusted blood
 Erythematous tender plaque with adherent "curdled milk"
like material on the hard palate and tongue Fine crackles on
both lung bases
 (+) undocumented fever, weight loss, anorexia, generalized
body weakness, morning fatigue
 No history of easy bruisability, epistaxis, gum bleeding,
menorrhagia, menometrorrhagia
 No history of hematuria, bubbly urine, jaundice
 No pallor or jaundice
 No joint pain/swelling
 No photosensitivity
 No history of recurrent abortions, preeclampsia or ec lampsia
 Anemia (Hemoglobin 101g/L, Hematocrit 0.31) Leukopenia
(WBC 2.52)
 Elevated ESR (110)
 +2 Albuminuria on urinalysis, no sugar or acetone
 No casts on urinalysis
 Normal sinus rhythm on ECG
 Pneumonitis, left lower lobe on chest x-ray
 No pleural effusion
ADMITTING
 Psoriasis vs. Tinea Corporis
 Apthous ulcers, buccal area
 To consider Systemic Lupus Erythematosus
 Hospital Care Associated Pneumonia
 Anemia, etiology to be determined

COURSE IN THE WARD

MRI
 Acute infarct, body and splenium of corpus callosum, right
corona radiata, bilateral centrum semiovale and bilateral
subcortical vertex.
EEG
 Mild generalized slowing of the background activity
 Mild intermitted slowing over the right centro-parietal region
The fdlowing laboratories COURSE INwere also requested
Antinuclear An abnormal titer of ANA by immuncifluorescence or an
antibodies equivalent assay at any point in time in the absence of
drugs known to Induce ANAs
 Any combination of 4 of 11 criteria, well documented at any
time during an individual's history, makes it likely that the
patient has SLE. (Sensitivity and specificity are 95% and 75%,
respectively.)
 Antinuclear antibodies (ANA) are positive in >98% of patients
during the course of disease

INTERPRETATION OF CLINICAL MANIFESTATIONS

FINAL DIAGNOSIS  Establish the severity and potential reversibility
 Systemic Lupus Erythematosus  Permanent complete remissions (absence of symptoms with
 Acute infarct, body and splenium of corpus callosum, right no treatment) are rare.
corona radiata, bilateral centrum semiovale and bilateral  Systemic symptoms, particularly fatigue and
subcortical vertex myalgias/arthralgias, are present most of the time
 Oral candidiasis, resolved
MUSCULOSKELETAL MANIFESTATIONS
SYSTEMIC LUPUS ERYTHEMATOSUS  Intermittent polyarthritis
 Soft tissue swelling and tenderness in joints, most commonly
in hands, wrists and knees
 Myalgia
 Ischemic necrosis of the bone

CUTANEOUS MANIFESTATIONS
 Lupus dermatitis can be classified as:
 Discoid lupus erythematosus (DLE)
 Systemic rash
 Subacutecutaneous lupus erythematosus (SCLE)
 "Others"

DISCOID LUPUS ERYTHEMATOSUS

RISK FACTORS  Roughly circular with slightly raised, scaly hyperpigmented
 Female sex is permissive for SLE erythematous rims and depigmented, atrophic centers in
 Estrogen containing oral contraceptives Hormone which all dermal appendages are permanently destroyed
replacement  Can be disfiguring
 Gene predisposition (T REX-I)  Incidence 20% among patients with SLE
 Exposure to ultraviolet light  Treatment: Topical or locally injected qlucocorticoids and
 Infections systemic antimalarials
 Epstein Barr Virus SYSTEMIC RASH
 Tobacco smoking  Photosensitive, slightly raised erythema
 Prolonged occupational exposure to silica  Worsening of this rash often accompanies flare of systemic
disease
DIAGNOSIS
Diagnostic Criteria for Systemic Lupus Erythematosus SUBCUTANEOUS LUPUS ERYTHEMATOSUS
Malar rash Fixed erythema, fiat or raised, over the malar eminences
 Scaly red patches similar to psoriasis, or circular flat red-
Discoid rash Erythematous circular raised patches with adherent
rimmed lesions.
keratotic scaling and follicular Pugging; atrophic scarring
may occur  Exquisitely photosensitive
Photosensitivity Exposure to ultraviolet light causes rash  Most have antibodies to Ro (SS-A)
Oral ulcers Includes oral and nasopharyngeal ulcers, observed by
physician RENAL MANIFESTATIONS
Arthritis Nonerosive arthntis of two or more peripheral joints,  Nephritis is the most serious complication
with tenderness, swelling, or effusion  Renal biopsy is useful
Serositis Pleuntis or pencardrtis documented by ECG or rub or
 Dangerous proliferative forms of glomerular damage (ISN Ill
evidence of effusion
and IV) usually have microscopic hematuria and proteinuria
Renal disorder Protainuna >0.5 g/d or 1r3+, or cellular casts
Neurologic Seizures or psychosis without other causes
(>500 mg per 24 h)
disorder  Approximately one-half develop nephrotic syndrome, and
Hematologic Hemolytic anemia or leukopenla (<40001tit) or most develop hypertension.
disorder lymphopsnia (<1500/tit.) or  Aggressive immunosuppression is indicated unless 90% of
thranbocytopenia(<100,000/ol) In the absence of glomeruli have irreversible damage
offending drugs
Immunologic Anti-dsDNA, anti-Sm, and/or anti-phospholipod disorder
  Leukopenia, almost always consists of lymphopenia not
granulocytopenia that rarely predisposes to infections
 Thrombocytopenia

GASTROINTESTINAL MANIFESTATIONS
 Nausea, vomiting, diarrhea
 Increases in serum aminotransferase (AST) and alanine
aminotransferase (ALT)
 Vasculitis involving the intestine may be life threatening
Perforations, ischemia, bleeding and sepsis are frequent
complications

OCULAR MANIFESTATIONS
 Sicca syndrome, nonspecific conjunctivitis Retinal vasculitis
and optic neuritis are serious manifestations
 Complications of glucocorticoid therapy include cataracts and
glaucoma

NERVOUS SYSTEM MANIFESTATIONS

 Seen in 5-15%
 19 different clinical manifestations
 No single simple diagnostic test
 Investigation of choice will vary with presentation It should
be determined whether they are caused by a diffuse process
or vascular occlusive disease
 The most common manifestation of diffuse CNS l upus is
cognitive dysfunction including difficulties with memory and
reasoning
 Headache, seizure, psychosis

TEST FOR AUTOANTIBODIES

VASCULAR OCCLUSION
 Transient ischemic attacks, stroke, myocardial infarction
 Antiphospholipid antibodies are associated with
hypercoagulability and acute thrombotic events Chronic
disease is associated with accelerated atherosclerosis
 The most important autoantibodies to detect are ANA as the
test is positive in >95% of patients, usually at the onset of
symptoms
 Increasing quantities of anti-dsDNA herald a flare, particularly
of nephritis and vasculitis
 Anti-Sm antibodies, although specific, do not correlate with
disease activity or clinical manifestations
 aPL identify patients at increased risk for venous or arterial
clotting, thrombocytopenia and fetal loss

TREATMENT
PULMONARY
 Pleuritis with or without pleural effusion  There is no cure for SLE and complete sustained remissions
are rare
 Pulmonary infiltrates also occur as a manifestation of SLE
 Patients will endure some adverse effects from treatment
 Life threatening pulmonary manifestations include interstitial
inflammation leading to fibrosis, shrinking lung syndrome and
NON-LIFE THREATENING DISEASE
intra-alveolar hemorrhage
 Analgesics
CARDIAC  NSAlDs
 Pericarditis  Acetaminophen
 Most serious cardiac manifestations are myocarditis and  Antimalarials
fibrinous endocarditis of Libman-Sacks
LIFE THREATENING DISEASE
 Endocardial involvement can lead to valvular insufficiencies
 Systemic glucocorticoids (0.5-1 mg/kg per day PO or 1000mg
HEMATOLOGIC of methylprednisolone sodium succinate IV daily for 3 days
followed by 0.5-1 mg/kg of daily prednisone or equivalent
 Most frequent hematologic manifestation is anemia, usually
normochromic normocytic
 Cytotoxic or immunosuppressive agents added to
glucocorticoids are recommended to treat serious SLE
 Cyclophosphamide
 Mycophenolate mofetil
 Azathioprine
 OTHER CONNECTIVE TISSUE DISEASE Pathology
(Dra. Saguil-Sy online class, 2021)
 Salivary gland biopsies show benign lymphoepithelial
1. Scleroderma proliferation and infiltration
2. Dermatomyositis / Polymyositis  Helper T cells predominant B cell activation
3. Sjogren's syndrome  Hypergammaglobulinemia and circulating antibody
4. Mixed connective tissue disease Clinical Findings
 Dry eyes/mouth
Scleroderma
 Tubular dysfunction
 A rare, common in women
Diagnostic test
 Multisystemic disorders
 ESR
 Characterized by skin thickening and vascular abnormalities
 Polycloned hypergammaglobulinemia
 Anemia
a) CREST syndrome - limited skin fibrosis of distal extremities
 ANA and RF (+) in 65-90%
b) Diffuse disease — patients with skin abnormalities extending
to the proximal extremities  Anti SSA/SSA antibodies
c) Localized disease - manifest as patches (morphea) or band Treatment
like  Lubricating eyedrops
Etiology - Unknown  Saliva substitute
Laboratory:
 Anti Scl 70 Dermatomyositis/ Polymyositis
 ANA  Are idiopathic inflammation
 Anticentromere  Myopathy
 RFT  Characterized by proximal muscle weakness
Early dermal changes includes:  Unknown etiology
 T-cell infiltrates  Associated with HLADR3
 Collagen accumulation with fibrosis Fibrosis of small to Pathology
medium size blood vessel  Mononuclear cell infiltrates
 Proliferation of sub-intimal tissue  Predominantly lymphocytes
Cardinal Findings  CD8 and T cell infiltration
 Raynaud's phenomenon  Increase in cytokines
 Skin thickening Esophageal dysmotility
 Arthralgia
 Cardiopulmonary (restrictive pulmonary disease) (PAH)
 Renal involvement — hypertension
 Eyes/mouth
 Hypothyroidism
Therapy
 Raynaud's phenomenon —vasodilators
 Hypertensive — ACE inhibitor
 Corticosteroids early disease
Clinical Manifestation
Mixed Connective Tissue Disease  Muscle – proximal symmetrical muscle weakness
 Skin – gottron papules, heliotrope rash, V neck/Shawl sign
 Scleroderma
 Cardiac – dysrhythmia
 SLE
 PM Diagnostic test
 RA  CPK
 EMG – myopathic motor unit potentials, low amplitude
 High levels of antibodies to UI-RNP
fibrillation
 Presents early Raynauds phenomenon
 Biopsy
 Good response with glucocorticoid
 MRI
 Autoantibodies: ANA (+) 50-80%, anti-Jo1
Sjogren’s Syndrome
 ESR, CRP
 Chronic autoimmune syndrome characterized by lymphocytic
infiltration of lacrimal and salivary glands with consequent Treatment:
dry eyes and dry mouth  Glucocorticoid
 Etiology — unknown  MTX
 Demography  Physical Therapy
o Middle age
o Predominant of female
 FIBROMYALGIA PHARMACOLOGIC TX
(Dra. Saguil-Sy online class, 2021)
 Antidepressants: balanced serotonin reuptake inhibitors
 characterized by chronic widespread musculoskeletal pain, o Amitryptiline
stiffness, paresthesia, disturbed sleep, and easy fatiguability o Duloxetine
along with multiple painful tender points, which are widely o Milnacipran
distributed  Anticonvulsants: ligands of the alpha -2delta subunit of
voltage gated calcium channels
PREVALENCE o Gabapentin
 3.4% in women and 0.5% in men o Pregabalin
 Most prevalent in women >50, and increases with age.

PATHOGENESIS
a) Disturbed sleep
b) Low levels of serotonin
c) Endocrine problem
d) Psychological abnormalities

MANIFESTATIONS
a) Musculoskeletal aching and stiffness and fatigue
b) Low back pain which may radiate to the buttocks and legs
c) Tightness in the neck and across upper posterior shoulders
d) Pain may begin in one region before becomes widespread

Potential tender points in fibromyalgia

The American College of Rheumatology 2010 criteria for the

classification of fibromyalgia
 does not require a tender-point count
 patients are assessed by
o the widespread pain index (WPI) - divides the body
into 19 regions and scores how many regions are
reported as painful
o a symptom severity score (SS) - assesses severity of
fatigue, unrefreshing sleep, and cognitive
symptoms
 provides a severity scale for associated fibromyalgia
symptoms
 A patient satisfies diagnostic criteria for fibromyalgia if the
following 3 conditions are met:
 widespread pain index (WPI) 27 and symptom severity (SS)
scale score >5 or WPI 3 - 6 and SS scale score ž9.
 symptoms have been present at a similar level for at least 3
months.
 the patient does not have a disorder that would otherwise
explain the pain

NONPHARMACOLOGIC TX
 Relieve anxiety
 Educate PX
 Physical conditioning
 Activities that promote improved physical fxn with relaxation
 VASCULITIS o Wegeners's granulomatosis
(Dra. Saguil-Sy online class, 2021)  Lower respiratory tract chronic granulomatous
Definition vasculitis inflammation
 Inflammatory infiltration of vessel walls with damage to  Necrotizing, pauci-immune glomerulonephritis
mutual structures  Associated with anti-proteinase 3
 Effort must be made to determine the cause o Churg-Strauss syndrome
 Known as allergic granulomatosis and angitis
Primary Vasculitis
 Also affects medium-sized vessels
 Autoimmune causes  Affects lungs and skin arteries Extravascular
 Classified scheme in 1993 Chapel Hill Concensus Conference granulomatosis
o Large  Associated in approximately 50% of cases with
o Medium anti-myeloperoxidase
o Small o Microscopic Polyangiitis
Classification of Vasculitis  Systemic vasculitis similar to Wegener's
 Large vessels granulomatosis
o Takayasu's arteritis  Anti-MPO antibodies
o Giant cell arteritis
 Medium vessels Secondary Vasculitis
o Polyarteritis nodosa
o Kawasaki disease  Non auto-immune cause of vasculitis
o Primary angiitis of the central nervous system  Etiologies:
 Small vessels o Medications
o Infections
LARGE VASCULITIDES o Malignancies
Takayasu's arteritis Pathophysiology
 Granulomatous inflammation affects the aorta and its main  Poorly understood
branches  Increased antigen load
 May involve all or just portion of vessels  Decreased clearance efficiency by the reticuloendothelial
 Typically in women from Japan with pulseless disease system Decreased solubility
 Pathogenic immune complexes fix complement
Giant Cell Arteritis  Lead to intense inflammation
 Granulomatous arteritis in cranial branches arising from
Diagnosis
aortic arch
 Assign the patient's signs and symptoms to a particular
 Also affects medium-sized vessel vessels size category
 Involved the temporal artery  Determine features within the category best fit the patient
 Associated with polymyalgia rheumatica
Clinical Presentation
 Seen in patient >50 years old, erythrocyte sedimentation rate
(ESR)  Most common constitutional symptoms:
o Fatigue
MEDIUM VASCULITIDES o Malaise
Polyarteritis nodosa (PAN) o Fever
 Necrotizing systemic vasculitis affecting both medium and o arrthralgias
small muscular arteries  Several clinical feature strongly suggest the presence of
 Without glomerulonephritis vasculitis:
 Associated with hepatitis B o Purpura
 Involved skin nodules, mononeuritis multiplex, orchitis and o Mononeuritis multiplex
mesenteric artery o Pulmonary-renal involvement
Kawasaki disease Differential Diagnosis
 Can affect large and small vessels Mostly in children  Embolic disorders
 Predilection for coronary artery  Infections
 Associated with mucocutaneous lymph node syndrome  SLE and amyloidosis
Primary angitis of the central nervous system  Ergots, cocaine and amphetamines
 Malignancies
 Rare granulomatous isolated in the leptomeninges
 Thrombocytopenia and myelodysplastic syndromes
SMALL VESSEL VASCULITIDES
 Subdivided into the presence or absence of immunoglobulin Diagnostic Testing
within the vessels  Laboratory
 Presents of immune complexes in vessels o CBC, creatinine, ESR and C-reactive protein (CRP),
o Henoch-Schonlein purpura hepatitis and HIV serologies, urinalysis and urine
o Cryoglobulinemic vasculitis toxicology screen Antinuclear antibodies (ANA)
o Connective tissue disease-associated vasculitis o Complement (cryoglobulinemia, hypocomplementemic
 Absence of immune complexes vessels urticarial vasculitis and vaculitis associated with SLE
 Associated with antineutrophil cytoplasmic antibodies o ANCA directed to PR3 by ELISA
  Imaging WEGENER'S GRANULOMATOSIS (WG)
o Chest x-ray
Clinical Presentation
o Sinus CT
 Initial Phase
o ECG
 Chronic inflammation usually in the upper airways.
o Angiogram
 Sinusitis as initial symptoms in >50% patients
 Diagnostic procedures
 May also occur in the oral cavity, retrobulbar space or airway
o Biopsy of affected tissue
obstruction
Treatment  Symptoms: Myalgia and arthralgia
 Goal of treatment:
o Induce remission of disease HENOCH-SCHONLEIN PURPURA
o Maintain less toxic immnusuppressant to prevent  Defined as a vasculitis with lgA-dominant immune deposits affecting
small vessels, including capillaries, venules and arterioles
relapses
o Based on type and severity of clinical Epidemiology
manifestations  Annual incidence is 14 cases per 100,000 people
o Immunosuppresant is the mainstay for treatment  Majority of patient are children less than 10 year old. Mean
 Medications age at presentation is 6 More common in male
o Glucocorticoids  Most common in the fall and winter months
o Cyclophosphamide  Often after respiratory infection
o Methotrexate, azarthioprine and mycophenola te Pathophysiology
mofetil
 Characterized by deposition of lgA-dominant immune
o Rituximab
complexes
Complications  With resultant complement activation
 Inadequately treated may lead to permanent endorgan  Leukocytoclastic vasculitis
damage and death  Skin and gastrointestinal manifestation are result of
 Relapse after induction treatment is unfortunately common, immunecomplex driven inflammation
but re-treatment usually leads to a good response
Clinical Presentation
TAKAYASUS’S ARTERITIS  Colicky abdominal pain associated with nausea and vomiting
Pathophysiology  Lower extremity arthritis
 Cause is unknown  Skin lesions
 Focal panarteritis affecting large vessels and major branches  Bloody diarrhea and palpable purpura
 Subsequent stenosis and aneurysm formation  Boys may present with orchitis
 Rare: headache or seizures
Clinical Presentation  Upper respiratory tract infection
 Divided to three monophasic stages BEHCET'S DISEASE
1. Phase 1 "prepulseless" inflammatory phase. Symptoms:  Systemic vasculitis characterized by recurrent aphthous oral and
fever, malaise, arthralgias and weight loss genital ulcers and uveitis
2. Phase 2 —vessel inflammation manifesting as vessel pain and  Associated and may present problem as skin lesions, joint
tenderness complaints, vascular disease and neurologic manifestation
3. Phase 3 "burn-out" or fibrotic stage. Lead to ischemic  Unique among vasculitis in its ability to affects vessels of any size
symptoms Clinical Presentation
Clinical Presentation  Erythema nodosum is observed in approximately 50% of
 Common findings in PE: patients in the course of disease and is more common in
o Pulselessness females
o Unequal brachial blood pressures Physical Examination
o Subclavian/carotid bruits  Oral aththosis
o Carotidynia o Lesions are typically round or oval shaped, painful,
 Hypertension 2 to 10 mm in size with sharp erythematous
Giant Cell Arteritis (GCA) border, a necrotic whitecentered based, and a
 Also known as temporal arteritis yellow pseudomembrane
 Affects the second to fifth order aortic branches Characterized by o Occur in gingiva, tongue, buccal and labial
granulomatous inflammation in vessels walls membrane
 Most common primary form of vasculitis among adults o Heal within approximately 10 days
 Patient with reasonable suspicion of GCA should be immediately o Tend to recur frequently
started won high-dose oral or intravenous glucocorticoid therapy to
 Ocular findings
suppress related blindness
 Once visual compromise has started, recovery in the affected eye o Anterior uveitis
rarely occurs even with aggressive treatment o Retinal disease
 Cutaneous manifestations
POLYMYALGIA RHEUMATICA
o Erythema nodusum
 Seen in individual >50 years old
 Manifest by symmetric pain and stiffness in the muscles for at least 4 o Pseudofolliculitis and acneiform nodules
weeks Closely related to GCA o Genital ulcers
 Respond very well to low-dose glucocorticoid therapy o Pathergy test