18 Jun 2021

1/7/21: Diffuse Alveolar Hemorrhage

PGY3 Semi Han presented a case of a 34 year old male with history of SLE (diagnosed in 2008), complicated by lupus nephritis, Raynaud’s, history of multiple pneumonias and latent TB who presented to ED for 2 days of productive cough and dyspnea on exertion.  +anterior b/l chest pain with taking deep breaths.  Patient reports sore throat and fatigue.  He denies any fevers, chills, sick contacts, headaches, hemoptysis, abdominal pain or diarrhea.  Home medications includes prednisone 5mg/4mg QOD, Plaquenil 200mg qday 3-4x weekly, mycophenolate mofetil 500mg BID intermittently.  The patient is a former smoker, drinks 7 beers/day, denies other drug use.

Vitals: T 36.8, HR 133, RR 24, BP 149/99, O2 sat 89% on RA but improved to 96% in 4LNC

Physical Exam significant for 2/6 holosystolic murmur heard at LL sternal border, diffuse crackles in all lung fields, worse in lower fields, increased work of breathing on 4L NC, normal abd exam an 1+ edema to b/l LE to knees

CBC: WBC 11.1> Hb 12.7/Hct 38.6 <Plt 198 (MCV 82.6, RDW 13.8%)

CMP:

Na 139/K 4.3/Cl 107/HCO3 22/BUN 14/Cr 1.08<Glucose 110

ALP 61>T.protein 5.5/Albumin 2.6/AST 28/ALT 11/Tbili 0.8/Dbili 0.2

Ca 8.2, Mg 2.0, Phos 3.2

Coags: PT 14.5, INR 1.4, PTT 30

Infectious workup initiated, with the following findings:

  • BCx negative, UCx negative
  • Flu/RSV/COVID negative
  • AFB smear and MTB PCR negative, Quantiferon gold negative

Pulm workup:

  • VBG: 7.33/46/27/23
  • ABG: 7.2/62/55/24, FiO2 100%
  • D-dimer: 1.84 mcg/mL (normal <0.49)

Rheum workup:

  • CRP elevated at 115.4
  • ESR elevated at 91
  • ANA negative, dsDNA 1:320
  • C3 low at 49, C4 low at 4.9
  • ANCA negative
  • APLS labs negative

CXR shows R>L ill defined hazy opacities predominantly peripherally and at the bases.

CTPA does not show pulmonary embolism.  Development of extensive bilateral prior oral and perihilar patchy opacities.  This is associated with some areas of consolidation.

Bronchoscopy was performed, showing the aliquots were progressively bloodier and darker, suggesting DAH.

Treatment: Patient was started on solumedrol 1g IV x3 days and Cellcept was initiated.  He was also given cyclophosphamide x1 and patient was discharged to with prednisone 60mg qday and continued on plaquenil 200mg qday.

Teaching points:

  1. Primary lung disease involved in lupus can include but not limited to: acute lupus pneumonitis, chronic ILD, pleural effusion, thromboembolic disease, PAH, DAH, shrinking lung disease
  2. DAH has been reported to complicate 2-5% of all cases of SLE. It is rare but life threatening complication of SLE.  It is classically reported as a neutrophilic capillaritis with destruction of alveolar septae and infiltration of haemosiderin-laden macrophages.
  3. Presenting sx of DAH: rapid onset of dyspnea, +/- hemoptysis, cough, fever. Can have acute drop of Hb and low C3 levels on labs.
  4. Treatment: Corticosteroids, cyclophosphamide, plasmapheresis, IVIg, Mycophenolate, Rituximab, stem cell transplantation, ECMO for refractory hypoxia even on mechanical ventilation. Also treat for infection – start on broad spectrum antibiotics

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