PMID- 34765369
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20211113
IS  - 2168-8184 (Print)
IS  - 2168-8184 (Electronic)
IS  - 2168-8184 (Linking)
VI  - 13
IP  - 10
DP  - 2021 Oct
TI  - Something Out of Nothing: A Rare Case of Pulmonary Renal Syndrome With 
      Pauci-Immune Glomerulonephritis and Diffuse Alveolar Hemorrhage With Negative 
      Serologies.
PG  - e18614
LID - 10.7759/cureus.18614 [doi]
LID - e18614
AB  - BACKGROUND: Pauci-immune crescentic glomerulonephritis (CrGN) is one of the most 
      common etiologies of rapidly progressive glomerulonephritis. This condition 
      presents with crescentic glomerulonephritis with little or no immunoglobulin 
      staining and negative serological workup aside from a positive antineutrophil 
      cytoplasmic autoantibody (ANCA). Typically, patients with pauci-immune CrGN have 
      an underlying systemic small vessel vasculitis, but in rare cases, it presents 
      without any known vasculitis or ANCA. Pauci-immune ANCA negative CrGN is often 
      strictly isolated to the kidneys. In this case, we present a patient with ANCA 
      negative, pauci-immune CrGN with severe diffuse alveolar hemorrhage. CASE 
      PRESENTATION: A 66-year-old Hispanic woman with a past medical history of 
      controlled hypertension presented with fatigue and dysphagia. On admission, her 
      vital signs were significant for hypoxia on room air, and her physical exam was 
      remarkable for crackles bilaterally. The initial laboratory results revealed 
      anemia (hemoglobin 5.2 g/dL), hyperkalemia (6.3 mmol/L), elevated creatinine 
      (4.50 mg/dL, with a baseline of 0.9mg/dL). Urinalysis showed moderate blood and 
      urine protein (200 mg/dL). Urine microscopic examination showed 25-50 RBCs 
      seen/high power field. The patient was admitted to ICU due to hypoxia, a computed 
      tomography scan of the chest/abdomen/pelvis was obtained and revealed multifocal 
      pulmonary consolidations. A blood transfusion was ordered. The patient began to 
      have hemoptysis and subsequent bronchoscopy showed diffuse alveolar hemorrhage. 
      ICU team proceeded to intubate her as the hemorrhage continued to worsen. Further 
      workup revealed a positive anti-nuclear antibodies (ANA) of 1:40, but otherwise 
      negative serologies including myeloperoxidase (MPO)-ANCA, glomerular basement 
      membrane antibody, and anti-double stranded DNA. Kidney biopsy showed necrotizing 
      glomerulonephritis with crescents and negative immunofluorescence. She was 
      diagnosed with pauci-immune ANCA-negative vasculitis with associated diffuse 
      alveolar hemorrhage and nephritis based on these results and was started on 
      pulse-dose steroids. The patient was started on intravenous (IV) high-dose 
      cyclophosphamide, which helped improved the overall clinical condition 
      significantly. After creatinine began trending down and urine output improved, 
      the patient was discharged on a regimen of daily oral cyclophosphamide and 
      steroid taper. Patient oxygen requirements decreased and she was sent home with 
      supplemental oxygen while requiring 3L/min of oxygen. CONCLUSION: Pauci-immune 
      and ANCA-negative glomerulonephritis with concurrent diffuse alveolar hemorrhage 
      is exceptionally rare. In this situation, medical management relied on clinical 
      evidence from similar populations in the use of steroids and cyclophosphamide. 
      This case report aims to shed more light on the clinical progression and 
      management of this condition. Here we present a case of pulmonary-renal syndrome 
      with biopsy-proven glomerulonephritis but without ANCA positive serologies.
CI  - Copyright (c) 2021, Wang et al.
FAU - Wang, Lucas Jiaxue
AU  - Wang LJ
AD  - Internal Medicine, Methodist Dallas Medical Center, Dallas, USA.
FAU - Collazo-Maldonado, Roberto
AU  - Collazo-Maldonado R
AD  - Nephrology, Methodist Dallas Medical Center, Dallas, USA.
LA  - eng
PT  - Case Reports
DEP - 20211008
PL  - United States
TA  - Cureus
JT  - Cureus
JID - 101596737
PMC - PMC8572681
OTO - NOTNLM
OT  - anca negative
OT  - diffuse alveolar hemorrhage
OT  - gross hematuria
OT  - pauci-immune crescentic glomerulonephritis
OT  - small vessel vasculitis
COIS- The authors have declared that no competing interests exist.
EDAT- 2021/11/13 06:00
MHDA- 2021/11/13 06:01
PMCR- 2021/10/08
CRDT- 2021/11/12 07:05
PHST- 2021/10/08 00:00 [accepted]
PHST- 2021/11/12 07:05 [entrez]
PHST- 2021/11/13 06:00 [pubmed]
PHST- 2021/11/13 06:01 [medline]
PHST- 2021/10/08 00:00 [pmc-release]
AID - 10.7759/cureus.18614 [doi]
PST - epublish
SO  - Cureus. 2021 Oct 8;13(10):e18614. doi: 10.7759/cureus.18614. eCollection 2021 
      Oct.