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Connective Tissue Disease

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by

LT Ong

on 2 June 2011

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Transcript of Connective Tissue Disease

Connective Tissue Diseases Heterogenous disorders
Share certain common features Inflammation of skin, joints and other structures rich in connective tissue
Altered patterns of immunoregulation Production of autoantibodies
Abnormalities of cell-mediated immunity Systemic Lupus
Erythematosus Scleroderma Dermatomyositis Constitutional Fatigue
Malaise
Fever
Weight loss Cutaneous Butterfly (malar)
rash
Photosensitivity
Discoid lesions
Oral ulcers
Alopecia
Raynaud's
phenomenon Sharply demarcated pallor resulting
from the closure of digital arteries Digital cyanosis of the fingertips in a patient
with primary Raynaud phenomenon Tightening of skin
of the face and
extremities
(sclerodactyly)
Telangiectasis
Calcinosis
Raynaud's
phenomenon Fatigue
Malaise
Arthralgia
Myalgia Tightening of the skin in the face, with a characteristic beaklike facies and paucity of wrinkles. Sclerodactyly with digital ulceration, loss of skin creases, joint contractures, and sparse hair. Muscle weakness
(symmetric, proximal) Gottron's sign
Heliotrope rash
Shawl & V sign
Erythroderma
Periungual abnormalities
Mechanic's hands
Psoriariform changes
in scalp
Flagellate erythema
Calcinosis An erythematous, scaly eruption over the extensor surfaces of the metacarpophalangeal joints and digits in a patient with dermatomyositis. A reddish-purple eruption on the upper eyelid (the heliotrope rash), accompanied by swelling of the eyelid in a patient with dermatomyositis. This is the most specific rash in DM, although it is only present in a minority of patients. Erythema that resembles the shawl sign but is seen in a variety of other cutaneous areas, including the malar region and the forehead. Multiple areas of capillary loop dilatation Periungual erythema A roughening and cracking of the skin of the tips and lateral aspects of the fingers, resulting in irregular, dirty-appearing lines that resemble those of a manual laborer Changes in the scalp resembling psoriasis Erythematous linear streaks,
probably induced by scratching
pruritic skin. Arthritis Inflammatory
Migratory
Assymmetric
Non-erosive
Only few joints
affected Patient with long-standing lupus has developed subluxation at the MCP joints and swan neck deformities of her fingers. These deformities are reducible (probably being due to lax tendons) and x-rays reveal no erosions or cysts, both of which differentiate these findings from those in rheumatoid arthritis. True, inflammatory
arthritis uncommon Polyarthritis Haematologic Anaemia (haemolytic)
Neutropenia
Thrombocytopaenia
Lymphadenopathy
Splenomegaly
Venous/arterial
thrombosis Cardiopulmonary Pleuritis
Pericarditis
Myocarditis
Endocarditis
(Libman-Sacks) Pulmonary
hypertension
Interstitial lung
disease
Pericarditis
Myocarditis
Arrythymias Interstitial lung disease
Myocarditis Renal Nephritis Gastrointestinal Peritonitis
Vasculitis Oesophageal
hypomotility
Incompetence of
LES Weakness of the striated
muscle (upper 1/3)
lead to dysphagia,
nasal regurgitation,
aspiration Neurologic Organic brain
syndromes
Seizures
Psychosis
Cerebritis Hypertension
Renal crisis/failure Opthalmologic Keratoconjunctivitis
sicca Antinuclear Antibodies
Full transcript