Pulmonary-Renal Syndrome in ICD10

Additional Info

• This refers to a syndrome of lung and kidney involvement. It is NOT a disease, it is a syndrome that has numerous causes and multiple possible manifestations both in the lungs and the kidneys.
• Most commonly, BUT NOT ALWAYS, it is due to one of another autoimmune disease
  • The two most common causes are: Wegener's granulomatosis and Goodpasture syndrome (hypersensitivity angiitis)
  • However, other autoimmune diseases that can cause it include:
    • Systemic lupus erythematosis (SLE, lupus)
    • Scleroderma or CREST syndrome (systemic sclerosis)
    • Collagen-vascular overlap syndrome -- including Mixed Connective Tissue Disease (MCTD)
    • Collagen-vascular disease, NOS
    • Necrotizing vasculopathy/vasculitis, NOS
    • Dermatomyositis/polymyositis
    • Microscopic polyangiitis/polyarteritis
    • Churg-Strauss syndrome (eosinophilic granulomatosis with polyangiitis (EGPA), allergic granulomatous angiitis)
    • and other auto-immune disorders
  • There are also non-autoimmune causes, most of which are immune-mediated reactions to infections.
• The lung manifestations are various and may include: hypoxemia, pulmonary hemorrhage, infiltrates
• The renal manifestations are various and may include: renal failure (acute or subacute or even chronic), hematuria
• In the case that a clear-cut pulmonary-renal syndrome is present, but the underlying disease CAUSING it is unknown you should:
  • If possible, wait for the cause to be found and list THAT.
  • To identify the pulmonary and renal manifestations, EVEN IF THE CAUSE IS CLEAR, combine them together (e.g. Hemorrhage, respiratory sites, NOS AND Nephritic syndrome, rapidly progressive)