Collagen vascular disease

In a class of diseases known as autoimmune disorders, the body's immune system attacks its own tissues. Some of these diseases are similar to each other. They may involve arthritis and inflammation of arteries in the tissues. People who developed these disorders were previously said to have "connective tissue" or "collagen vascular" disease. We now have names for many specific conditions such as:

Ankylosing spondylitis
Ankylosing spondylitis
Ankylosing spondylitis (AS) is a chronic form of arthritis. It mostly affects the bones and joints at the base of the spine where it connects with t...
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Dermatomyositis
Dermatomyositis
Dermatomyositis is a disease that involves muscle inflammation and a skin rash. Polymyositis is a similar inflammatory condition that also involves ...
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Polyarteritis nodosa
Polyarteritis nodosa
Polyarteritis nodosa is a serious inflammatory blood vessel disease. The small and medium-sized arteries become swollen and damaged.
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Polymyositis
Psoriatic arthritis
Psoriatic arthritis
Psoriatic arthritis is a joint problem (arthritis) that often occurs with a skin condition called psoriasis.
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Rheumatoid arthritis
Rheumatoid arthritis
Rheumatoid arthritis (RA) is a disease that leads to inflammation of the joints and surrounding tissues. It is a long-term disease. It can also aff...
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Scleroderma
Scleroderma
Scleroderma is a disease that involves the buildup of fibrous tissue in the skin and elsewhere in the body. It also damages the cells that line the ...
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Systemic lupus erythematosus
Systemic lupus erythematosus
Systemic lupus erythematosus (SLE) is an autoimmune disease. In this disease, the immune system of the body mistakenly attacks healthy tissue. It c...
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Mixed connective tissue disease
Relapsing polychondritis
Vasculitis

When a specific disease cannot be diagnosed, more general terms may be used. These are called undifferentiated systemic rheumatic (connective tissue) diseases or overlap syndromes.

Open References

References

Graf J. Overlap syndromes. In: Firestein GS, Budd RC, Gabriel SE, Koretzky GA, McInnes IB, O'Dell JR, eds. Firestein & Kelley's Textbook of Rheumatology. 11th ed. Philadelphia, PA: Elsevier; 2021:chap 91.

Teegavarapu SP, Mims MP. Lymphocytosis, lymphocytopenia, hypergammaglobulinemia, and hypogammaglobulinemia. In: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Basic Principles and Practice. 8th ed. Philadelphia, PA: Elsevier; 2023:chap 50.

Dermatomyositis - heliotrope eyelids - illustration
This photograph demonstrates the sign heliotrope eyelids in which the eyelids develop a brown (violaceous - rather than red) color. Heliotrope eyelids and Gottron's papules on the knuckles are characteristic findings in dermatomyositis.
Dermatomyositis - heliotrope eyelids
illustration
Polyarteritis - microscopic on the shin - illustration
This is a picture of microscopic polyarteritis on the shin. The term polyarteritis means that many blood vessels are inflamed. These nodules are located just below the skin's surface (subcutaneous), are barely felt when pressing on the skin (palpable), and are tender. The skin is typically red (erythematous).
Polyarteritis - microscopic on the shin
illustration
Systemic lupus erythematosus rash on the face - illustration
This is a photo of a systemic lupus erythematosis rash on the face. Lupus erythematosis often produces a butterfly rash or malar rash. Typically, the rash also appears on the nose.
Systemic lupus erythematosus rash on the face
illustration
Sclerodactyly - illustration
The most classic symptom of scleroderma is a type of skin tightening called sclerodactyly. The initial stages of the disease involves swelling of the fingers. Later, as the connective tissue becomes fibrotic, skin on the fingers and toes becomes hard and shiny. The fingers can become difficult to bend and can form contractures due to the severe tightening of the skin.
Sclerodactyly
illustration
Rheumatoid arthritis - illustration
The affect of rheumatoid arthritis can progress to the degree that it is crippling. Deformities distinctive to late-stage rheumatoid arthritis such as ulnar deviation of the bones of the hands, or swan-neck deviation of the fingers occur because muscles and tendons on one side of the joint may overpower those on the other side, pulling the bones out of alignment.
Rheumatoid arthritis
illustration

Dermatomyositis - heliotrope eyelids - illustration
This photograph demonstrates the sign heliotrope eyelids in which the eyelids develop a brown (violaceous - rather than red) color. Heliotrope eyelids and Gottron's papules on the knuckles are characteristic findings in dermatomyositis.
Dermatomyositis - heliotrope eyelids
illustration
Polyarteritis - microscopic on the shin - illustration
This is a picture of microscopic polyarteritis on the shin. The term polyarteritis means that many blood vessels are inflamed. These nodules are located just below the skin's surface (subcutaneous), are barely felt when pressing on the skin (palpable), and are tender. The skin is typically red (erythematous).
Polyarteritis - microscopic on the shin
illustration
Systemic lupus erythematosus rash on the face - illustration
This is a photo of a systemic lupus erythematosis rash on the face. Lupus erythematosis often produces a butterfly rash or malar rash. Typically, the rash also appears on the nose.
Systemic lupus erythematosus rash on the face
illustration
Sclerodactyly - illustration
The most classic symptom of scleroderma is a type of skin tightening called sclerodactyly. The initial stages of the disease involves swelling of the fingers. Later, as the connective tissue becomes fibrotic, skin on the fingers and toes becomes hard and shiny. The fingers can become difficult to bend and can form contractures due to the severe tightening of the skin.
Sclerodactyly
illustration
Rheumatoid arthritis - illustration
The affect of rheumatoid arthritis can progress to the degree that it is crippling. Deformities distinctive to late-stage rheumatoid arthritis such as ulnar deviation of the bones of the hands, or swan-neck deviation of the fingers occur because muscles and tendons on one side of the joint may overpower those on the other side, pulling the bones out of alignment.
Rheumatoid arthritis
illustration

A Closer Look

Talking to your MD

Self Care

Tests for Collagen vascular disease

Review Date: 1/25/2023

Reviewed By: Neil J. Gonter, MD, Assistant Professor of Medicine, Columbia University, NY and private practice specializing in Rheumatology at Rheumatology Associates of North Jersey, Teaneck, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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Health Library

Collagen vascular disease

Autoimmune disorders

Ankylosing spondylitis

Dermatomyositis

Polyarteritis nodosa

Psoriatic arthritis

Rheumatoid arthritis

Scleroderma