Pulmonary-Renal Syndrome in ICD10

Background

• 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
    • 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
• As this diagnosis is a syndrome and not a disease, and it involves two organ systems, you should ALSO code the manifesting symptoms as Combined ICD10 codes; for example this might be Hemorrhage, respiratory sites, NOS AND Nephritic syndrome, rapidly progressive
• if the cause (likely one of the conditions listed above) is known, list its code as the same Combined ICD10 codes as the symptoms
• if the cause is not known by the end of the ward stay, then as usual, code the manifestations, which may be signs or symptoms