Code first and use additional: the etiology and manifestation rule

Some conditions must be coded as a pair, in a set order. The code first and use additional notes are the manual telling you which comes first and why.

Published 2026-08-30 · by M.R. Sterling

Sooner or later you meet a condition that will not sit in a single code. The manual tells you to record two codes, in a particular order, and it is very firm about which comes first. This is the etiology and manifestation rule, and the little notes that enforce it, code first and use additional, confuse people until the logic clicks. Then it becomes one of the more satisfying parts of coding.

Etiology and manifestation

Some conditions are really two linked things. There is an underlying cause, the etiology, and there is the effect it produces in a particular body system, the manifestation. A disease and a specific complication it brings on. The manual's position is that both matter, and that their relationship matters, so both are coded, and the order is not left to you.

The convention is that the underlying cause is sequenced first and the manifestation second. Cause before effect. That order tells anyone reading the record what drove what.

The two notes that tell you

The manual signals this with instructional notes attached to the codes themselves.

  • A code first note sits with a manifestation code. It is telling you this code cannot stand alone, and that the underlying cause must be coded and sequenced ahead of it.
  • A use additional code note sits with an underlying-cause code. It is telling you that you should also code a manifestation, sequenced after it, when one is present.

The two notes are the same instruction seen from opposite ends. One points backward to the cause that must come first. The other points forward to the manifestation that should follow. When you see either, the manual is describing a pair.

These notes are not suggestions. When a code carries a code first note, using it on its own, without the underlying cause ahead of it, is an incomplete and incorrect record.

Why the order is not cosmetic

Sequencing is not tidy-mindedness. The order of codes carries meaning, and downstream systems read it. In an inpatient stay the first-listed diagnosis has outsized weight, and getting cause and effect the wrong way round can misrepresent what the admission was actually about. Cause first is how the record says "this is the driver, and this is what it led to".

How to spot the situation

You will not have to guess. The manual is explicit: the notes appear right at the code, in the tabular list, exactly where you are looking when you assign it. The skill is not detection, it is trusting the note and not talking yourself out of it. When a code says code first, find the underlying cause and put it ahead. When a code says use additional, check whether a manifestation is documented and, if so, add it after.

Where to go next

Conventions like this are the difference between codes that are individually plausible and a record that is actually correct, and sequencing is where careful coders earn their reputation. Conventions in Practice works through the manual's instructional notes on real cases, and Selection & Sequencing covers the wider question of which code leads and why.

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