What are connective tissue diseases?
A connective tissue disease is any disease that
affects the parts of the body that connect the structures of the body together.
Connective tissues are made up of two proteins: collagen and elastin. Collagen
is a protein found in the tendons, ligaments, skin, cornea, cartilage, bone, and
blood vessels. Elastin is a stretchy protein that resembles a rubber band and is
the major component of ligaments and skin. When a patient has a connective
tissue disease, the collagen and elastin are inflamed. The proteins and the body
parts they connect are harmed.
What are the different types of connective tissue
There are several different types of connective tissue
diseases. They can be inherited or caused by environmental factors. Connective
tissue diseases include, but are not limited to:
Rheumatoid arthritis (RA) — Rheumatoid arthritis is
one of the most common connective tissue diseases and can be inherited. RA is an
autoimmune disease, meaning the immune system attacks its own body. In this
systemic disorder, immune cells attack and inflame the membrane around joints.
It also can affect the heart, lungs, and eyes. It affects many more women than
men (an estimated 71% of cases).
Scleroderma—an autoimmune condition that causes scar
tissue to form in the skin, internal organs (including the GI tract), and small
blood vessels. It affects women three times more often than men throughout life,
occurring at a rate of 15 times greater for women during childbearing years.
Wegener's Granulomatosis—A form of vasculitis
(inflammation of the blood vessels) that affects the nose, lungs, kidneys and
Churg-Strauss Syndrome—A type of autoimmune vasculitis
that affects cells in the blood vessels of the lungs, gastrointestinal system,
skin, and nerves.
Systemic Lupus Erythematosus Lupus (SLE)—A disease
that can cause inflammation of the connective tissue in every organ of the body,
from the brain, skin, blood, to the lungs. It is nine times more common in women
Microscopic Polyangitis—An autoimmune disease that
affects cells in blood vessels in organs throughout the body. It is a rare
Polymyositis/Dermatomyositis—A disease characterized
by inflammation and degeneration of the muscles. When the condition also affects
the skin, it is called dermatomyositis.
How do connective tissue diseases affect the lungs and
One of the most serious impacts of connective tissue
disease is on the pulmonary system. The types of connective tissue disease
mentioned above can involve the lungs and affect breathing.
Here is an overview:
Rheumatoid arthritis—Although RA mainly affects the
joints, it can cause serious complications that affect the lungs. These include
painful breathing due to inflammation of the lung lining and shortness of breath
because inflammation of the lining causes fluid to accumulate in the lungs. RA
can also cause lung nodules (spots) called rheumatoid nodules to form. These
usually do not cause symptoms. A more common effect of RA on the lungs is
scarring of the tissue due to the inflammation. Symptoms of this many include
shortness of breath, a chronic cough, weakness and fatigue. Treatment for
pulmonary complications of rheumatoid arthritis includes removing excess fluid
from the lungs and using medications to suppress the immune system that will
decrease the inflammation in the lungs.
Scleroderma—About 80% of all patients with scleroderma
have pulmonary complications. Without treatment, lung involvement is the leading
cause of death from scleroderma. Scleroderma can cause inflammation of the lung
tissue, lung scarring, and injury to the blood vessels in the lungs. Symptoms of
these include a feeling of breathlessness and fatigue. Treatment includes the
use of immune-suppressing medications, regular exercise, and the use of
supplemental oxygen if needed.
Wegener's Granulomatosis—This disease mostly affects
the upper respiratory tract (sinuses and nose), as well as the breathing tubes
and the lung tissue by causing inflammation and damage to the blood vessels in
those organs. Pulmonary symptoms include a cough that might include bloody
phlegm, chest discomfort with or without shortness of breath, nose bleeds, and a
persistent runny nose. Treatment for this condition is primarily the use of
anti-inflammatory and immune suppressing medications.
Churg-Strauss Syndrome—This condition that affects
blood vessels in the lungs can have serious breathing complications. It first
presents with asthma-like symptoms, including shortness of breath and the
inability to catch your breath. Other symptoms can include coughing up blood.
The lungs can also develop scarring as well as accumulate fluid. Treatment
includes the use of immunosuppressant drugs, most usually prednisone. This is
very effective in treating the syndrome.
Systemic Lupus Erythematosus Lupus (SLE)—More than 50%
of patients with SLE have pulmonary complications. Pleurisy (inflammation of the
lining of the lung) is the most common problem. Other complications include the
build-up of fluid around the lung and scarring of the lung tissue. SLE can also
cause a condition called lupus pneumonitis, an inflammation of the lungs that
can be chronic. Symptoms of these conditions include shortness of breath, chest
pain, difficulty breathing and coughing. Lupus pneumonitis may cause a fever.
Treatment includes immunosuppressant drugs, rest, and careful monitoring by your
Microscopic Polyangitis—This condition is very similar
to, but rarer than, Wegener's Granulomatosis and causes inflammation of the
blood vessels in the lungs. Pulmonary problems are similar, including bloody
cough and shortness of breath. Treatments are also anti-inflammatory and
Polymyositis/Dermatomyositis —Polymyositis can cause
scarring of the lungs, which causes symptoms such as shortness of breath and
coughing. The disease can also affect the muscles surrounding the lungs, which
can make it difficult to take deep breaths and can rarely lead to respiratory
failure. Treatments include the use of immunosuppressant drugs, oxygen and
Call your doctor right away if you experience any of
the above symptoms or if you are already diagnosed with a connective tissue
disease and are experiencing breathing problems. With proper treatment, many
pulmonary complications of connective tissue disease can be managed along with
other complications of the disease.
The Merck Manual for Health Care Professionals. Mixed Connective Tissue
Disorders and The Merck Manual for Health Care Professionals.
Connective Tissue Disorders in Children.
American Journal of Medicine
www.amjmed.com Accessed 8/5/2011
National Heart Lung and Blood Institute. Diseases and Conditions Index: Types of Vasculitis
Scleroderma Foundation. Lung Fibrosis in System Sclerosis.
www.scleroderma.org Accessed 8/5/2011
Vasculitis Foundation. Microscopic Polyangiitis.
www.vasculitisfoundation.org Accessed 8/5/2011
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