Learning objectives
LEARNING OBJECTIVES
Pulmonary-renal syndromes (also known as lung-kidney and pulmo-renal syndromes) are a set of rare conditions with varying etiology,
brought together by the combined manifestation of diffuse alveolar haemorrhage and glomerulonephritis.
This work aims to provide a thorough and comprehensive theoretical review of the most common representatives of this group,
with emphasis on the medical imaging aspect of diagnosis.
Hence,
treatment options are not discussed.
Conditions encompassed in this review are:
ANCA(anti-neutrophil cytoplasmic antibody) associated vasculitides:
granulomatosis with polyangiitis (Wegener’s granulomatosis),
eosinophillic granulomatosis with...
Background
BACKGROUND
The pulmonary-renal syndrome is defined by the combination of diffuse alveolar haemorrhage and rapid progressive glomerulonephritis (RPGN) [1,2,11].
Diffuse alveolar haemorrhage comprises the triad of hemoptysis,
diffuse alveolar infiltrates and low hematocrit.
However,
clinical presentation can be variable (slight cough,
progressive dyspnea,
manifest hemoptysis).
Symptoms need not present simultaneously.
Rapid-progressive glomerulonephritis manifests in quick loss of renal function (within a few days/weeks) and the presence of a nephritic sediment with deformed erythrocytes of a glomerular origin and possibly red cell casts [11].
Due to...
Findings and procedure details
FINDINGS AND PROCEDURE DETAILS
As previously mentioned,
pulmonary-renal syndromes manifest with diffuse alveolar haemorrhage and glomerulonephritis.
The latter’s appearances on imaging vary with the evolution of the disease.
Often the only demonstrable abnormality is a change in size - always affecting both kidneys symmetrically.
In the acute phase the kidneys are enlarged due to swelling,
with no evident papillary/calyceal abnormality.
Later,
in the chronic phase,
the kidneys are small,
with smooth margins and normal pelvicalyceal systems.
The amount of renal sinus fat may appear excessive...
Conclusion
CONCLUSION
Pulmo-renal syndromes are a group of rare diseases with overlapping pathological and imaging manifestations.
Due to their rarity and quite often non-specific imaging findings,
it is essential that a radiologist has sufficient grasp on their potential pulmonary and renal manifestations,
as to avoid missing a suspect diagnosis.
Only by making a comprehensive correlation with a patient’s clinical picture,
their history and laboratory results can the diagnosis be reasonably narrowed down,
and in some cases even established before biopsy confirmation.
The primary imaging modality with...
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