Connective To Emergency PDF

Connective Tissue Disorder
Emergencies
DR.Rajesh Gubba
• All the Connective tissue disorders are multi system disorders.
•
Rheumatoid Arthritis Ankylosing Behcets syndrome Reiters syndrome
Spondylitis
Cause of Death
Pathognomic
manifestation
C/F emergency
Other features
IOC & HLA association
Treatment
Treatment of
Emergency
• Deformities seen with hand involvement
• Swan Neck deformity  Hyperextension of PIP joint.
• Button hole deformity  Hyperextension of DIP joint.
4
Spondylitis
Cause of Death Ischemic heart disease # Rigid osteoporotic Pulmonary artery Cardiac Conduction
spine vasculitis – PA defects
aneurysms
Pathognomic Small Joint Spine & SI joint Oral ulcers Triad – A,U,C
manifestation involvement
C/F emergency Chest pain Severe pain – Spine Dyspnea Giddiness & Syncopal
FND attack
Other features
IOC & HLA association Anti CCP Sr.Matrix metallo HLA b5,51 HLA B27
HLA DR3,4 proteinase 3
Treatment NSAIDS,Steroids NSAIDS Colchicine & Analgesics,

Methotrexate TNF alpha(-)/IL 17 A(-) Steroids(Topical) Tetracyclines,Steroids
Treatment of
Emergency
Spondylitis
Cause of Death
Pathognomic
manifestation
C/F emergency
Other features
Treatment
Treatment of
Emergency
Spondylitis
aneurysms
FND attack
Other features

Treatment of
Emergency
Spondylitis
Cause of Death
Pathognomic
manifestation
C/F emergency
Other features
Treatment
Treatment of
Emergency
Spondylitis
aneurysms
FND attack
Other features

Treatment of
Emergency
12
• Eye involvement may be one of the most devastating complications of Behcet
disease.
• Posterior uveitis, in essence a retinal venulitis, may lead to the insidious

destruction of large areas of the retina before the patient becomes aware of visual
problems.
• Anterior uveitis, associated with the triad of photophobia, blurred vision, and a red
eye, is intensely symptomatic.
13
What is the complication of Anterior
Uveitis?
• This complication may lead to a hypopyon, the accumulation of pus in
the anterior chamber.
• If not treated properly with mydriatic agents to dilate the pupil and
corticosteroid eyedrops to diminish inflammation, the anterior uveitis
may lead to synechial formation between the iris and lens, resulting in
permanent pupillary distortion.
15
Spondylitis
Cause of Death
Pathognomic
manifestation
C/F emergency
Other features
Treatment
Treatment of
Emergency
Spondylitis
aneurysms
FND attack
Other features

Treatment of
Emergency
SLE Sjogrens syndrome Gouty Arthrits Marfans syndrome
Cause of Death
Pathognomic
manifestation
C/F emergency
Other features
Treatment
Treatment of
Emergency
Cause of Death Cardiac involvement > CAD (Thrombo Cardiovascular death Pneumothorax
Renal failure embolism) Aortic dissection
Pathognomic MC- Next slide Dry eyes & Dry mouth 1st MTP joint Flexible joints
C/F emergency CHF Chest pain Chest pain Sudden onset dyspnea
Chest pain
Other features Extraglandular Ghents criteria

features
IOC & HLA association HLA-DRB1 Synovial fluid analysis FBN 1 gene mutation
Treatment Steroids NSAIDS, Steroids, Col Labetolol

Allopurinol,Febuxostat Aortic root stenting
Treatment of Diuretics TLT

Emergency
SLE
• MC presentation of SLE is fatigue, malaise, fever and arthralgia:
95%.
• This is followed by hematological and cutaneous manifestations.
20
Clinical Features of SLE
• Malar rash Butterfly rash (MC acute SLE rash)
• Fixed erythema, flat or raised, over the malar eminences and sun
exposed parts.
21
• Discoid rash
• Erythematous circular raised patches with adherent keratotic scaling
and follicular plugging; atrophic scarring may occur.
22
• Photosensitivity (Ro/SS-A antibody)
• Exposure to sunlight leads to rash/ sun burn
• Treatment: Hydroxychloroquine and sunscreen lotion
23
• Oral ulcers
• They are superficial small ulcers which are painless
and occur on hard palate, buccal cavity and
vermiform border.
24
• Synovitis
• This involves hands, wrists, knee j oints and is non-
erosive
Non-erosive arthritis is seen in cases of:

1. Rheumatic fever
2. Inflammatory bowel disease
3 . SLE
25
• Musculoskeletal manifestation in SLE :-
• Polyarthralgias and polyarthritis eventually occur in 90% of SLE patients.
• The arthritis initially involve the small joints in hands, knees and wrists.
• Joint deformities develop in only 10%.
• Erosions on joints X-rays are rare, their presence suggest a non lupus
inflammatory arthropathy such as rheumatoid arthritis
26
• Cardiovascular Features
• Myocarditis and pericarditis.
• Endocarditis (Libman-Sacks endocarditis).
• A higher risk of coronary artery disease compared to general population due to

increased risk of atherosclerosis and vasculitis.
27
• Cardiac involvement:
• MC involvement is pericarditis.
• Libman Sacks endocarditis is the characteristic feature of

cardiac SLE.
• Chest pain at rest due to irritation of phrenic nerve
• Upon auscultation: Pericardial friction rub
• IOC: ECG, ST elevation; Concave upwards in all leads except aVR.
• Rx of Libman Sacks endocarditis: Valvuloplasty.

28
• Lung involvement in SLE :-
• Pleuritis and pleural effusions (most common pulmonary manifestations

affecting almost 50% of patients).
• MC lung involvement in SLE: Pleuritis.
• Alveolar injury can also occur in the form of edema and hemorrhage.
• In some cases there is chronic interstitial fibrosis.
• Rare involvement is Shrinking lung syndrome and intraalveolar

hemorrhage 29
• Characterized by unexplained dyspnea, a restrictive
pattern on pulmonary function tests, and an elevated
hemidiaphragm.
• The chest radiograph reveals elevated diaphragms,

and pulmonary function tests shows reduced lung
volumes.
• This condition has been describe as "shrinking lung"

and is thought to be caused by diaphragmatic
myopathy
30
31
• Kidney involvement: Lupus nephritis
• Present with hematuria (cola-colored urine)
32
• Classification of Lupus Nephritis (International Society
ofNephrology and Renal Pathology Society)
• Class 1: Minimal Mesangial Lupus Nephritis
• Class II : Mesangial Proliferative Lupus Nephritis
• Class Ill : Focal Lupus Nephritis
• Class IV: Diffuse Lupus Nephritis
• Class V: Membranous Lupus Nephritis
• Class VI: Advanced Sclerotic Lupus Nephritis
33
• Investigations:
• a. Urine m/e- >3 RBC/HPF and presence of RBC casts
• b. Urine protein concentration: 0.5 - l g/24 hours
34
• d. IOC: For lupus nephritis kidney biopsy.
• Upon HPE, wire loop lesion is characteristic finding.
• Most severe stage of lupus nephritis: Stage IV (diffuse

lupus nephritis)
• Treatment: Hemodialysis if GFR < 30 mL
• Transplantation if GFR < 1 5 mL (ESRD).

35
• Psychosis/ Lupus Cerebritis:-
• Lupus antibody can cross (blood brain barrier) and can
cause cerebral edema and seizures.
• MC CNS manifestation: Cognition defect (antibody = Anti-

neuronal Ab)
• MC psychiatric manifestation: Psychosis [antibody=

AntiRNP ( ribonucleoprotein)Ab]
• Treatment: Haloperidol
• Spinal cord involvement in form of myelopathy 36

37
• Hematological manifestation:
• Autoimmune hemolytic anemia because antibodies
destroys own RBC.
• These are warm antibodies: IgG  active at 37°C
• On peripheral smear: Spherocytes seen
• IOC: Direct Coomb's test
• Treatment: Steroids
38
• Lymphopenia and thrombocytopenia is also seen.
• Immunological manifestations in SLE
• ANA > upper reference limits
• Anti-ds DNA
• Anti-Sm
• Antiphospholipid antibody
• Low serum complement
• Positive direct Coombs test
39
Cause of Death
Pathognomic
manifestation
C/F emergency
Other features
Treatment
Treatment of
Emergency
Chest pain

features


Emergency
42
• Extraglandular manifestation of sjogren's syndrome
• Arthralgias / arthritis - MC extraglandular manifestation in
primary Sjogren is arthralgia/arthritis.
• Least common involvement is myositis.
• Raynaud's phenomenon
• Lymphadenopathy
• Lung involvement
• Vasculitis
• Kidney involvement 43
• Liver involvement
• Lymphoma
• Splenomegaly
• Peripheral neuropathy
• Myositis
44
Cause of Death
Pathognomic
manifestation
C/F emergency
Other features
Treatment
Treatment of
Emergency
Chest pain

features


Emergency
Cause of Death
Pathognomic
manifestation
C/F emergency
Other features
Treatment
Treatment of
Emergency
Renal failure embolism) < Aortic dissection
Chest pain

features


Emergency
Scleroderma APLA syndrome Giant cell Arteritis Takayasu arteritis
Cause of Death
Pathognomic
manifestation
C/F emergency
Other features
IOC
Treatment
Treatment of
Emergency
Cause of Death P.HTN CAD Cardiovascular CAD
Pathognomic Skin involvement Recurrent abortions Head ache Pulseless disease

manifestation Raynauds
C/F emergency Dyspnea Arterial /Venous MI/CHF/Aortic MI

occlusion dissection
Other features EYE Other vessels

PMR
IOC Anti Centromere Anticardiolipin Biopsy MRI Aorta

Anti Topoisomerase Anti b2 Glycoprotein 1
Lupus Anticoagulant
Treatment Steroids Anticoagulants & Steroids Steroids
Aspirin
Treatment of PAH lowering drugs TLT

Emergency
55
56
57
58
Cause of Death
Pathognomic
manifestation
C/F emergency
Other features
IOC
Treatment
Treatment of
Emergency



PMR

Lupus Anticoagulant
Aspirin

Emergency
Cause of Death
Pathognomic
manifestation
C/F emergency
Other features
IOC
Treatment
Treatment of
Emergency



PMR

Lupus Anticoagulant
Aspirin

Emergency
63
Cause of Death
Pathognomic
manifestation
C/F emergency
Other features
IOC
Treatment
Treatment of
Emergency



PMR

Lupus Anticoagulant
Aspirin

Emergency

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Connective Tissue Disorder

IOC & HLA association

Treatment NSAIDS,Steroids NSAIDS Colchicine & Analgesics,

IOC & HLA association

Treatment NSAIDS,Steroids NSAIDS Colchicine & Analgesics,

IOC & HLA association

Treatment NSAIDS,Steroids NSAIDS Colchicine & Analgesics,

• Posterior uveitis, in essence a retinal venulitis, may lead to the insidious

IOC & HLA association

Treatment NSAIDS,Steroids NSAIDS Colchicine & Analgesics,

IOC & HLA association

Other features Extraglandular Ghents criteria

Treatment Steroids NSAIDS, Steroids, Col Labetolol

Treatment of Diuretics TLT

• This is followed by hematological and cutaneous manifestations.

Non-erosive arthritis is seen in cases of:

2. Inflammatory bowel disease

• Polyarthralgias and polyarthritis eventually occur in 90% of SLE patients.

• Joint deformities develop in only 10%.

• Endocarditis (Libman-Sacks endocarditis).

• A higher risk of coronary artery disease compared to general population due to

• Libman Sacks endocarditis is the characteristic feature of

• Chest pain at rest due to irritation of phrenic nerve

• Upon auscultation: Pericardial friction rub

• IOC: ECG, ST elevation; Concave upwards in all leads except aVR.

• Rx of Libman Sacks endocarditis: Valvuloplasty.

• Pleuritis and pleural effusions (most common pulmonary manifestations

• MC lung involvement in SLE: Pleuritis.

• In some cases there is chronic interstitial fibrosis.

• Rare involvement is Shrinking lung syndrome and intraalveolar

• The chest radiograph reveals elevated diaphragms,

• This condition has been describe as "shrinking lung"

• Present with hematuria (cola-colored urine)

• Class II : Mesangial Proliferative Lupus Nephritis

• Class Ill : Focal Lupus Nephritis

• Class IV: Diffuse Lupus Nephritis

• Class V: Membranous Lupus Nephritis

• Class VI: Advanced Sclerotic Lupus Nephritis

• b. Urine protein concentration: 0.5 - l g/24 hours

• Upon HPE, wire loop lesion is characteristic finding.

• Most severe stage of lupus nephritis: Stage IV (diffuse

• Treatment: Hemodialysis if GFR < 30 mL

• Transplantation if GFR < 1 5 mL (ESRD).

• MC CNS manifestation: Cognition defect (antibody = Anti-

• MC psychiatric manifestation: Psychosis [antibody=

• Spinal cord involvement in form of myelopathy 36

• These are warm antibodies: IgG  active at 37°C

• On peripheral smear: Spherocytes seen

• IOC: Direct Coomb's test

IOC & HLA association

Other features Extraglandular Ghents criteria

Treatment Steroids NSAIDS, Steroids, Col Labetolol

Treatment of Diuretics TLT

IOC & HLA association

Other features Extraglandular Ghents criteria

Treatment Steroids NSAIDS, Steroids, Col Labetolol

Treatment of Diuretics TLT

IOC & HLA association

Other features Extraglandular Ghents criteria

Treatment Steroids NSAIDS, Steroids, Col Labetolol

Treatment of Diuretics TLT

Cause of Death P.HTN CAD Cardiovascular CAD