Diffuse Alveolar Haemorrhage — DCE Clinical Case

Focused history — what you must establish

• The respiratory trajectory: onset and progression of dyspnoea, cough, haemoptysis (volume, frequency), exertional tolerance, and any preceding 'sinusitis' or 'pneumonia' that failed antibiotics — the failed-treatment story is the vasculitis signature [1].
• Upper-airway and systemic features: nasal crusting, bloody discharge, saddle-nose change, hearing loss, ear pain; fevers, weight loss, night sweats; rash; joint pains; eye symptoms; numbness or foot-drop (mononeuritis).
• Bleeding modifiers: anticoagulants, antiplatelets, NSAIDs; occupational exposures — as a gardener, hydrocarbon and solvent exposure matters because such exposures unmask the alveolar epitope in anti-GBM disease [7].
• Renal clues: frothy urine, dark or cola-coloured urine, nocturia, ankle swelling; baseline creatinine and prior urine results.
• The patient's frame: what he thinks is wrong, what he fears, his work and family situation — a long case is a person, not a syndrome.

Examination priorities

General inspection for pallor and respiratory distress; palpable purpura on the legs and buttocks (vasculitic, non-blanching, raised); nasal examination for crusting, septal perforation and the saddle-nose bridge of GPA; ears for effusion; eyes for scleritis; chest for diffuse fine crackles and signs of consolidation; cardiovascular examination for a flow murmur and — before finishing — urine dipstick for blood and protein with a request for microscopy, because the kidney completes the pulmonary-renal syndrome [1] [2].

Presentation template (deliver this to the examiner)

"Mr Ellery is a 54-year-old gardener with a 6-week systemic illness: progressive dyspnoea with one episode of haemoptysis, blood-stained nasal crusting, arthralgia and weight loss, on a background of a 38 g/L haemoglobin fall and a creatinine that has tripled. On examination he has palpable purpura, nasal crusting with early bridge change, diffuse bilateral crackles and a hypoxic room-air saturation; his urine dipstick shows blood and protein. This is a pulmonary-renal syndrome — diffuse alveolar haemorrhage with an active nephritic sediment — and granulomatosis with polyangiitis is my leading diagnosis. My immediate steps are serial bronchoalveolar lavage to confirm alveolar haemorrhage, same-day ANCA with PR3/MPO specificity, anti-GBM, ANA and complement, urine microscopy, and renal biopsy when stable; and because he is hypoxic with active capillaritis physiology, I would start pulse methylprednisolone now and plan rituximab induction." [1] [5]

Management — what you will actually do

1. Confirm DAH: bronchoscopy with serial BAL — progressively bloodier aliquots confirm it; haemosiderin-laden macrophages confirm bleeding beyond 48–72 hours; send cultures to exclude the infective mimic [1].
2. Same-day serology: ANCA ELISA with PR3 and MPO specificity, anti-GBM antibody, ANA/dsDNA, complement C3/C4; HIV and hepatitis serology before immunosuppression; urine microscopy for red-cell casts [5].
3. Start treatment on suspicion: pulse methylprednisolone 500–1000 mg IV daily for 3 days then a reduced-dose oral taper per PEXIVAS, plus rituximab 375 mg/m² weekly for 4 doses — the RAVE-established induction for severe AAV [3] [4].
4. Plasma exchange only if: refractory life-threatening DAH or double-positive anti-GBM/ANCA serology — PEXIVAS removed routine apheresis from ANCA vasculitis [4].
5. Renal biopsy once stable to confirm pauci-immune crescentic GN and grade reversibility; nephrology co-management of his creatinine trajectory [5].
6. Aftercare: PJP prophylaxis, bone protection, vaccination planning, smoking and solvent-exposure cessation advice, then maintenance rituximab 500 mg every 6 months once in remission — superior to azathioprine in MAINRITSAN — with structured relapse surveillance [6].

Probing questions

"Why did you reach for the bronchoscope rather than starting antibiotics and repeating the CT?" — "His picture is already 6 weeks old with a falling haemoglobin and renal involvement; another week of empiric antibiotics costs the diagnosis and possibly the kidney. Serial BAL is the test that confirms DAH and simultaneously samples for infection — it answers both questions in one procedure" [1].

"The anti-GBM result comes back positive as well as PR3-ANCA. Does that change anything?" — "Yes — double-positive disease behaves like anti-GBM acutely and like ANCA vasculitis long-term. I would add plasma exchange now for the anti-GBM component, and still plan maintenance immunosuppression because his relapse risk follows the ANCA pattern" [4] [5].

"He asks why he can't just have antibiotics and go home." — "I would explain that his immune system is attacking the small blood vessels of his lungs, kidneys and nose — that the bleeding and the kidney injury come from the same fire, and that antibiotics cannot reach it. The treatment calms that immune attack, works best started early, and then steps down to a gentler medicine that keeps it quiet" [2].

"What is his relapse plan?" — "Rituximab maintenance 500 mg six-monthly for at least 18–24 months, scheduled reviews with urinalysis, creatinine, CRP and blood count, ANCA titres as an adjunct rather than a trigger, and a written action plan — haemoptysis, cola-coloured urine or a sinus flare means urgent review, not the next routine slot" [6] [5].