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Systemic connective tissue inflammation syndrome (SCTIS)
The additional information. There are three main diseases due to the SCTIS: Systemic
lupus erythematosus (SLE), Scleroderma (SD), and Dermatomyositis (DM)
Complaints
Inflammatory joint disorders
1. Rest pain
2. Swelling of the joints;
3. Limitation of the function of joints;
Inflammatory muscle disorders
1. Usage pain.
2. Swelling of the muscles or Muscle atrophy;
3. Limitation of the function of joints
4. Weakness; Fatigue;
5. Morning stiffness;
6. Diplopia, Dysartria, Dysphagia, Breathlessness.
History
Non-specific symptoms of systemic illness
Inflammation of serous membranes
Symptoms of pleuritis, pericarditis
Inflammation of internal organs
• Lungs (breathlessness, cough, pneumorrhagia);
• Heart (chest pain, arrhythmia, breathlessness, palpitation, edema);
• Gastro-intestinal tract (dysphagia, stomach-ache, nausea, vomiting,
constipation, gastrointestinal bleeding).
Neurological disorder
Fatigue, headache, poor concentration, insomnia, and etc.
Present illness
1. There are acute, subacute or chronic onsets of SCTDS.
2. The previous infections or other provoking factors
3. SCTDS are life-long, with intermittent exacerbations and remissions.
SCTDS has progressive character.
4. Some patients have mild disease; in others it is more severe.
5. Previous treatment and its results
Life history
environmental hazards; the occurrence of Inflammatory joint disorders in
members of their family; allergies.
General Survey
1. Apparent State of Health: Acutely or chronically ill
2. Active, Passive or Enforced Posture
3. Weight loss
Physical Examination
Scleroderma
Skin changes
1. The initial phase of skin disease is characterized by non-pitting
oedema of the fingers. Subsequently, the skin becomes shiny and taut.
2. The flection contractures of the fingers are begun.
3. The face and neck are usually involved next, with thinning of the lips
and radial furrowing. The patient has the Immobile face.
For SLE
1. Photosensitivity. Skin rush as a result of unusual reaction to sunlight.
2. The classic butterfly (malar) facial rash is raised and painful or pruritic
and occurs in photosensitive distribution that spares the nasolabial
folds.
3. Discoid lupus lesions are characterized by hyperkeratosis and
follicular plugging and may cause scarring alopecia if present on the
scalp.
For DM
1. Heliotrope rash is a violaceous discoloration of the eyelid in
combination with periorbital oedema. Similar rashes occur on the
upper back, chest and shoulders (“shaw” distribution).
2. Gottron’s papules are scaly erythematous plagues or papules
occurring over the extensor surface o the proximal and distal
interphalangeal joins.
Other skin manifestation:
Periungual nail-fold capillaries; Teleangiectasia; livedo reticularis; Erithema
multiform; Urticaria; Erithema nodosum; Hyper- and depegmentation
Hair and nail lesions
•Loss of hair;
•Trophic nail changes
Vascular disorders
•Digital ulceration (necrosis)
Mucous membrane lesions
•Oral ulcers. Oral or nasopharyngeal ulceration, may be painless.
•Cheilitis
Lymphatic nodes lesions
•Generalized lumphadeopathy
Inflammatory joint disorders
•There may be only migratory arthralgias, but sometimes the non-
erosive polyarthritis develops. These disorders are symmetrical, and
usually involve peripheral joints.
•Deformities of joints may rarely be present in chronic current.
Inflammatory muscle disorders
•There are ether muscle atrophy or polymyositis.
Inflammatory bone disorders
•Osteolysis
•Pathological bone fracture
Inflammatory periarticular lessons
•Tenosynovitis
Inflammation of serous membranes
•Serositis: pleuritis, pericarditis, perihepatitis, episplenitis
Neurological disorders
•Visual hallucinations, chorea, organic psychosis, transverse myelitis and
lumphocytic meningitis.
Inflammation of internal organs
•Lungs (alveolitis, lung fibrosis, pulmonary hypertension and etc.);
•Heart (myocarditis, sterile endocarditis, venous thromboembolism);
•Gastro-intestinal tract (hepatomegalia, gastritis, enteritis, colitis and etc.)
•Splenomegaly, Kidney (hypertension), Thyroid gland (thyroiditis)
Lab tests
1. Total blood count [anaemia, leucopenia or leucocytosis, lymphopenia,
thromocytopenia]; increased ESR,
2. Urine stick test [persistent proteinuria > 0,5 g/day], Urine microscopy
[erythrocytes, cellular casts (red cell, granular or tubular)]
3. Serum proteins (inflammatory results); CRP - positive test;
4. LE cells,
5. Abnormal immunofluorescent test for antinuclear antibodies (ANA); Anti-
DNA antibodies; antibodies to Sm antigen; antiphospholipid antibodies.
6. Positive urine oxiproline test;
7. Increased serum muscle enzymes level
8. Biopsies of skin, kidney, muscles
Investigations
Imaging Studies
1. X-ray image; (osteoporosis, ossifluence of distal phalanges, Calcified
deposits in skin or muscles)
Systemic lupus erythematosus
Scleroderma
Dermatomyositis

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Systemic Connective Tissue Inflammatory Syndrome Lecture 4

  • 1. Systemic connective tissue inflammation syndrome (SCTIS) The additional information. There are three main diseases due to the SCTIS: Systemic lupus erythematosus (SLE), Scleroderma (SD), and Dermatomyositis (DM) Complaints Inflammatory joint disorders 1. Rest pain 2. Swelling of the joints; 3. Limitation of the function of joints; Inflammatory muscle disorders 1. Usage pain. 2. Swelling of the muscles or Muscle atrophy; 3. Limitation of the function of joints 4. Weakness; Fatigue; 5. Morning stiffness; 6. Diplopia, Dysartria, Dysphagia, Breathlessness. History
  • 2. Non-specific symptoms of systemic illness Inflammation of serous membranes Symptoms of pleuritis, pericarditis Inflammation of internal organs • Lungs (breathlessness, cough, pneumorrhagia); • Heart (chest pain, arrhythmia, breathlessness, palpitation, edema); • Gastro-intestinal tract (dysphagia, stomach-ache, nausea, vomiting, constipation, gastrointestinal bleeding). Neurological disorder Fatigue, headache, poor concentration, insomnia, and etc. Present illness 1. There are acute, subacute or chronic onsets of SCTDS. 2. The previous infections or other provoking factors 3. SCTDS are life-long, with intermittent exacerbations and remissions. SCTDS has progressive character. 4. Some patients have mild disease; in others it is more severe. 5. Previous treatment and its results
  • 3. Life history environmental hazards; the occurrence of Inflammatory joint disorders in members of their family; allergies. General Survey 1. Apparent State of Health: Acutely or chronically ill 2. Active, Passive or Enforced Posture 3. Weight loss Physical Examination
  • 4. Scleroderma Skin changes 1. The initial phase of skin disease is characterized by non-pitting oedema of the fingers. Subsequently, the skin becomes shiny and taut. 2. The flection contractures of the fingers are begun. 3. The face and neck are usually involved next, with thinning of the lips and radial furrowing. The patient has the Immobile face.
  • 5. For SLE 1. Photosensitivity. Skin rush as a result of unusual reaction to sunlight. 2. The classic butterfly (malar) facial rash is raised and painful or pruritic and occurs in photosensitive distribution that spares the nasolabial folds.
  • 6. 3. Discoid lupus lesions are characterized by hyperkeratosis and follicular plugging and may cause scarring alopecia if present on the scalp.
  • 7. For DM 1. Heliotrope rash is a violaceous discoloration of the eyelid in combination with periorbital oedema. Similar rashes occur on the upper back, chest and shoulders (“shaw” distribution).
  • 8. 2. Gottron’s papules are scaly erythematous plagues or papules occurring over the extensor surface o the proximal and distal interphalangeal joins.
  • 9. Other skin manifestation: Periungual nail-fold capillaries; Teleangiectasia; livedo reticularis; Erithema multiform; Urticaria; Erithema nodosum; Hyper- and depegmentation
  • 10. Hair and nail lesions •Loss of hair; •Trophic nail changes
  • 13. Mucous membrane lesions •Oral ulcers. Oral or nasopharyngeal ulceration, may be painless. •Cheilitis Lymphatic nodes lesions •Generalized lumphadeopathy Inflammatory joint disorders •There may be only migratory arthralgias, but sometimes the non- erosive polyarthritis develops. These disorders are symmetrical, and usually involve peripheral joints. •Deformities of joints may rarely be present in chronic current.
  • 14. Inflammatory muscle disorders •There are ether muscle atrophy or polymyositis.
  • 15. Inflammatory bone disorders •Osteolysis •Pathological bone fracture Inflammatory periarticular lessons •Tenosynovitis Inflammation of serous membranes •Serositis: pleuritis, pericarditis, perihepatitis, episplenitis Neurological disorders •Visual hallucinations, chorea, organic psychosis, transverse myelitis and lumphocytic meningitis.
  • 16. Inflammation of internal organs •Lungs (alveolitis, lung fibrosis, pulmonary hypertension and etc.); •Heart (myocarditis, sterile endocarditis, venous thromboembolism); •Gastro-intestinal tract (hepatomegalia, gastritis, enteritis, colitis and etc.) •Splenomegaly, Kidney (hypertension), Thyroid gland (thyroiditis)
  • 17. Lab tests 1. Total blood count [anaemia, leucopenia or leucocytosis, lymphopenia, thromocytopenia]; increased ESR, 2. Urine stick test [persistent proteinuria > 0,5 g/day], Urine microscopy [erythrocytes, cellular casts (red cell, granular or tubular)] 3. Serum proteins (inflammatory results); CRP - positive test; 4. LE cells, 5. Abnormal immunofluorescent test for antinuclear antibodies (ANA); Anti- DNA antibodies; antibodies to Sm antigen; antiphospholipid antibodies. 6. Positive urine oxiproline test; 7. Increased serum muscle enzymes level 8. Biopsies of skin, kidney, muscles Investigations
  • 18. Imaging Studies 1. X-ray image; (osteoporosis, ossifluence of distal phalanges, Calcified deposits in skin or muscles)
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