Pulmonary manifestations in scleroderma: a review

Oyoo.G.O, Genga.E. "Pulmonary manifestations in scleroderma: a review.". In: ISSN.; 2014.


Background: Scleroderma is a chronic
multisystem autoimmune disease of
unknown aetiology. Scleroderma is
characterized by widespread obliterative
vasculopathy of small arteries and is
associated with varying degrees of tissue
fibrosis and multiple organ involvement.
Pulmonary disease is an important
component of SSc. It is estimated that
80% of patients with SSc have some
evidence of pulmonary disease. Systemic
sclerosis has the poorest prognosis
amongst rheumatology diseases with
the highest case-specific mortality of any
of the autoimmune rheumatic diseases
as well as causing major morbidity.
Objective: This article will review
pathogenesis, diagnosis and
management of pulmonary disease in
Data source: Literature review of
relevant published literature from both
Africa and the rest of the world.
Data synthesis: The pathogenesis of
lung disease in scleroderma involves
a variety of pathways, including
immunological/inflammatory activation
and vascular injury. The primary
cytokines responsible for the disease
are unknown but it is postulated that it
involves a complex interplay between
inflammatory, B lymphocyte antibody
production, oxidative stress and fibrotic
pathways. This leads to the activation
of lung fibroblasts by inflammatory and
fibrotic mediators. Lung fibroblasts play
a central role in the deposition of excess
intracellular matrix. This inflammatory
response leads to fibrosis and occurs in
the setting of vascular derangements.
The most common symptoms are dry
cough and dyspnea on exertion. The
high morbidity and mortality seen in
SSc is generally attributed to the two
major pulmonary manifestations of the
disease: interstitial pulmonary fibrosis, or
interstitial lung disease, and pulmonary
arterial hypertension. Exertional dyspnea
and dry cough are the most common
presenting symptoms in patients
with SSc who develop pulmonary
involvement Algorithm of diagnostic
procedures in these patients does not
Department of Clinical
Medicine and Therapeutics,
College of Health Sciences,
University of Nairobi, Kenya
Corresponding author:
Dr GO Oyoo. Email:
Review Article
differ considerably from the procedures
of any other interstitial lung disease. At
the current time, cyclophosphamide
remains the best studied therapeutic
agent although alternatives are actively
being evaluated. The pathogenesis of
pulmonary disease in scleroderma is
still an enigma and is being actively
researched. This will advance our
understanding of the disease and ability
to care for these patients.
Conclusion: Pulmonary complications
are common in SSc and are the leading
causes of death. Careful evaluation
by the clinician is warranted to detect
the presence of an ILD and to select
patients appropriately for consideration
of therapy. It is a major clinical challenge
largely due to the enigma of the disease
pathology as well as limited therapeutic
options available. This is compounded
by the perceived lack of evidence for
clinical effectiveness of those treatments
that are currently in use. Clinical trials
are underway and offer hope for novel
approaches to this mysterious and
often devastating manifestation of

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