CONNECTIVE TISSUE

DISRODERS
IMMUNE COMPLEX DISEASES INHERITED DISORDER

-deposition of immune Bone Skin, Cartilage, Blood

complexes causes connective Vessels
tissue damage - Marfan syndrome, Ehlers
- sterile inflammation - Danlos
predominantly of skin , joints,
blood vessels and kidney
- e.g. SLE and rheumatoid
arthritis
Systemic Lupus Erythematosus

Antiphospholipid Syndrome

Rheumatoid arthritis

Sjogren

Scleroderma
SYSTEMIC LUPUS
ERYTHEMATOSUS
+ loss of immune tolerance and persistent
autoantibody production ( antinuclear antibody,,
anti double stranded DNA antibody)
+ Initial presentation – polyarthralgias, fatigue,
photosensitive skin rash
- lupus nephritis
SYSTEMIC LUPUS
ERYTHEMATOSUS
SYSTEMIC LUPUS ERYTHEMATOSUS

+ THERAPY
- Long term anti malarial therapy – Hydroxychloroquine
- Exacerbation treated with steroids, in critical conditions with IV
methylprednisolone

- - chronic corticosteroid use is avoided, and steroid sparing therapy is initiated

( azathioprine, mycophenolate mofetil, methotrexate)
- - frequent monitoring every 3 to 4months
SYSTEMIC LUPUS ERYTHEMATOSUS
and PREGNANCY
MATERNAL RISKS

- Preeclampsia,
- sponatenous abortion,
placental abruptio PRECONCEPTION
thromboembolic disease, COUNSELING
- post partum infection
( active renal diseasese
increases mortality)
SYSTEMIC LUPUS ERYTHEMATOSUS
and PREGNANCY
+ SLE FLARE
- Rate of flare 3% - 2nd trimester and 3rd trimester
- Those with active disease 6 mos before conception are at a
highest risk for flare during pregnancy ( 60%)
- Flare most commonly diagnosed when pregnant patient has a
NEW OR INCREASED RASH, lymphadenopathy, arthritis, fever
SYSTEMIC LUPUS ERYTHEMATOSUS
and PREGNANCY
Prepregnancy evaluation ( renal function)

Review of current medical regimen

MANAGEMENT Measurement of autoantibodies ( aPL,

anticardiolipin and anti B2 glycoprotein,
anti-Ro/SSA and anti-La/SSB

Co managed by rheumatologist and MFM

Serial fetal sonography at 18 weeks

onwards and fetal surveillance at 32
weeks
Systemic Lupus Erythematosus

Antiphospholipid Syndrome

Rheumatoid arthritis

Sjogren

Scleroderma
ANTIPHOSPHOLI PID SYNDROME

autoimmmune condition characterized clinically by thrombosis

and/or severe adverse pregnancy outcomes

+ - Confirmation with positive aPLs- lupus anticoagulant,

anticardiolipin antibody, and anti-β2 glycoprotein-I
PATHOGENESIS
+ aPls bond to the antigens expressed by
phospholipid binding protein

+ APAs are theorized to cause pregnancy loss by

thrombosis of placental vessels, interference with
coagulation factors (reduce levels of annexin V),
inhibition of proliferation of trophoblasts,
complement activation,
ANTIPHOSPHOLI
PID SYNDROME
 MANAGEMENT
PRECONCEPTION COUNSELING TREATMENT OBSTETRIC MANAGEMENT

- Evaluate underlying renal  Combination of Heparin and - Serial sonography for fetal
disease and hypertension LDA growth and AFI from 16-18
weeks
- Pregancy prognosis can be >For APS with ≥3 unexplained
stratified according to patient consecutive pregnancy losses at - BP monitoring ( early detection
history and aPL status <10 weeks or ≥1 fetal loss >10 of GH)
weeks: Low-dose ASA and
> (+) LA, triple positivity of aPL prophylactic heparin - Antental surveillance at 32
antibodies  fetal death and early • For APS with VTE during the weeks or earlier if with
delivery for PES and placental current pregnancy: Therapeutic suspected FGR, materal
insufficiency despite treatment anticoagulation with heparin. hypertension
• For APS with VTE prior to
pregnancy: Prophylactic
anticoagulation with heparin
Systemic Lupus Erythematosus

Antiphospholipid Syndrome

Rheumatoid arthritis

Sjogren

Scleroderma
RHEUMATOID ARTHRITIS
- Inflammatory disease marked by chronic symmetrical
inflammatory arthritis of small and medium sized joints
- - peak onset 35- 55 years old
PATHOPHYSIOLOGY
+ Autoantibodies to citrullinated peptides
associated with complement activation
leads to SYNOVIAL MEMBRANE
INFLAMMATION marked by cellular
hyperplasia, accumulation of inflammatory
leukocytes with membrane thickening and
fibrin deposition joint damage
+  osteoclast formation leading to bone
degradation and cartilage damage
CLINICAL PRESENTATION
+ pain and stiffness in multiple joints,
proximal interphalangeal and
metacarpal phalangeal joints
+ - formation of rheumatoid nodules

- Laboratory testing – tests for ACPA

, RF , ESR and CRP
TREATMENT
+ control of inflammation and prevention of joint damage

- Glucocorticoids ( prednisone) maintenance medication

- Intraarticular steroids can be used
PREGNANCY MANAGEMENT
+ Serial sonography every 4- 8 weeks ( risks for FGR)
+ Monitor for gestational hypertension

+ RA flare occurs within firs three months posatpartum

Systemic Lupus Erythematosus

Antiphospholipid Syndrome

Rheumatoid arthritis

Sjogren

Scleroderma
SJOGREN
+ keratoconjunctivitis, sicca, arthritis
+ secondary to autommune disease SLE or RA

- Most common risk is neonatal lupus – (+) anti SS A and anti SS

B antibodies
- risk of preeclampsia, PROM, FGR and congenital
malformation
Systemic Lupus Erythematosus

Antiphospholipid Syndrome

Rheumatoid arthritis

Sjogren

Scleroderma
Systemis sclerosis
( Scleroderma)

+ hallmark is thickened hardened

skin characterized by marked
increased in collagen in the
dermis with hyalinization and
obliteration of small blood
vesells
+ progressive fibrosis of viscera
lungs, kidneys, GI tract
CLINICAL PRESENTATION
+ skin involvement beginning with edematous swelling and erythema of
fingers, hands and face thickening and hardening and loss of
appendicular hair, hyperpigmentation, digital ulcers
- classified
a. Limited Cutaneous SSc- skin sclerosis restricted to hands, distal
forearm, face and neck. (+) prominent vascular malformation
b. Diffuse Cutaneous SSc- affected skin chest, abdomen, upper arms
- develop significant organ damage caused by ischemic injury or
fibrosis
CLINICAL PRESENTATION

+ vascular dysfunction- Reynauds phenomenon

+ increased risk for venous thromboembolic disease
+ Dysphagia caused by esophageal dysmotility and
gastroesophageal reflux
+ Pulmonary involvement- interstitial lung disease and pulmonary
artery hypertension
+ cardiac- myocardial fibrosis, arrythmias, heart failure
PREGNANCY OUTCOME
+ risk of preterm birth and FGR
+ mortality renal and pulmonary disease
+ SSc renal crisis – malignant hypertension, acute renal failure,
proteinuria
MANAGEMENT
+ diffuse Ssc with cardiac pulmonary and renal involemevemet
should be COUNSELED AGAINST attempting pregnancy

+ Increased maternal surveillance with visits every 2 to 4 weeks in

first half of pregnancy, then 1 to 2 weeks thereafter
+ fetal growth monitoring and antenatal testing at 30-32 weeks
+ DELIVERY before term particularly with the diffuse type
- vaginal delivery is not CONTRAINDICATED
SLE

APAS

Rheumatoid arthritis

Sjogren
SYSTEMIC VASCULITIS
+ presence of inflammatory leukocytes in vessel walls with
reactive damage to mural strucutures
- loss of vessel integrity hemorrhage and tissue ischemia and
necrosis
- classification based on predominant size of the vessels
involved
SYSTEMIC VASCULITIS
+ disease is uncommon data on pregnancy and neonatal
outcome is limited
- reports higher pregnancy loss, preterm delivery

- counselling, pregnancy attempted when disease is in a

sustained remission
- immunosuppressive aganets ( cyclophosphamide) teratogenic
hence should be discontinued before pregnancy
ANTI RHEUMATIC DRUGS IN PREGNANCY
MEDICATIONS
Hydroxychloroquine SAFE in Pregnancy and Lactation
Sulfasalazine
Colchicine
Azathioprine
Prednisone -optimal dose < 20 mg daily during pregnancy
- undergo early screening for GDM
Tumor Necrosis Compatible with Pregnancy and lactation
Inhibitor - Discontinuation in the third trimester
( Certolizumab)
Methotrexate TERATOGENIC
Leflunomide - Cyclophosphamide use in the second and 3rd trimester for lif threatening
Cyclophosphamide disease
CONNECTIVE TISSUE
DISRODERS