CASE REPORT |
https://doi.org/10.5005/jp-journals-11006-0130 |
Unprecedented Encounter: A Rare Encounter of ANCA Vasculitis with Diffuse Alveolar Hemorrhage and Ileal Perforation
1Department of Critical Care Medicine, Bhailal Amin General Hospital, Vadodara, Gujarat, India
2Department of Internal Medicine, Bhailal Amin General Hospital, Vadodara, Gujarat, India
3Department of Pulmonology, Bhailal Amin General Hospital, Vadodara, Gujarat, India
4Department of Rheumatology, Bhailal Amin General Hospital, Vadodara, Gujarat, India
5Department of Nephrology, Bhailal Amin General Hospital, Vadodara, Gujarat, India
Corresponding Author: Bhavin Patel, Department of Critical Care Medicine, Bhailal Amin General Hospital, Vadodara, Gujarat, India, Phone: +91 9998979267, e-mail: drbhavin007@gmail.com
Received: 06 March 2024; Accepted: 12 July 2024; Published on: 27 August 2024
ABSTRACT
Aim and background: This case report aims to highlight the atypical presentation of ANCA-associated vasculitis, where the patient experienced simultaneous occurrences of diffuse alveolar hemorrhage (DAH) and ileal perforation. ANCA-associated vasculitis is an autoimmune disorder that can lead to inflammation and damage of small blood vessels, resulting in various clinical manifestations. DAH is a common and severe pulmonary complication, while ileal perforation is a rare and life-threatening gastrointestinal complication.
Case description: A 35-year-old male patient was admitted to the ICU with fever, dyspnea, and hemoptysis. He was diagnosed with MPO-positive ANCA vasculitis based on laboratory findings, and diagnostic tests. The patient’s condition rapidly deteriorated, requiring invasive ventilatory support. He was treated with a combination of cyclophosphamide, rituximab, and high-dose corticosteroids. During treatment, he developed abdominal pain, dyspnea, and hypotension, and was found to have a small bowel perforation on imaging. The patient underwent emergency exploratory laparotomy, and the perforation was surgically repaired.
Conclusion: This case highlights the atypical and severe manifestations of ANCA-associated vasculitis, with the occurrence of DAH and ileal perforation. The management of such complex cases requires a multidisciplinary approach, with prompt and aggressive treatment of the vasculitis and management of the complications.
Clinical significance: This case emphasizes the importance of clinicians being aware of the potential for rare and atypical manifestations of ANCA-associated vasculitis. Early recognition and prompt intervention are crucial to improve patient outcomes. The case also need for further research to better understand the pathophysiological mechanisms and establish optimal treatment protocols for these rare complications.
Keywords: ANCA vasculitis, Case report, Cyclophosphamide, Diffuse alveolar hemorrhage, Ileal perforation, Rituximab
How to cite this article: Patel B, Shah R, Patel D, et al. Unprecedented Encounter: A Rare Encounter of ANCA Vasculitis with Diffuse Alveolar Hemorrhage and Ileal Perforation. Indian J Crit Care Case Rep 2024;3(5):130–132.
Source of support: Nil
Conflict of interest: None
Patient consent statement: The author(s) have obtained written informed consent from the patient for publication of the case report details and related images.
INTRODUCTION
The case report describes a rare presentation of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, specifically involving diffuse alveolar hemorrhage (DAH) and ileal perforation. ANCA-associated vasculitis is a group of autoimmune disorders characterized by inflammation of small blood vessels, and it commonly affects the kidneys and lungs. However, the occurrence of DAH and ileal perforation in the context of ANCA-associated vasculitis is uncommon and poses diagnostic as well as management challenges.
CASE DESCRIPTION
A 35-year-old male patient was shifted to the intensive care unit (ICU) with a history of fever, dyspnea, and hemoptysis. He had paresthesia, (vasculitic) skin rash, leukocytosis with eosinophilia, raised immunoglobulin E (IgE) level, and diagnosed myeloperoxidase (MPO) positive ANCA vasculitis along with anti-glomerular basement membrane (GBM) antibody positive with other blood reports (Table 1). He had respiratory distress with hypoxia and was put on oxygen therapy. Pulmonologist and rheumatologist opinions were taken. The prognosis of the patient, along with the consequences and required treatment, was discussed with his relatives. The patient further worsened with respiratory distress and required intubation and invasive ventilatory support along with vasopressor support. Chest X-ray (Fig. 1) was suggestive of diffuse white opacity in the bilateral lung field. The patient was treated with ARDSnet protocol for ventilator support. Blood transfusion was given as per requirement. The patient was treated with cyclophosphamide followed by rituximab and higher antibiotics, along with antifungal treatment as a fungal marker in the form of galactomannan and beta-d-glucan were positive. DAH was decreased after the first dose of rituximab (which was given after all cultures including fungal culture were negative). He was treated with methylprednisolone pulse therapy for 3 days and cyclophosphamide pulse therapy (total two doses given). He was also given rituximab 500 mg in two doses and continued with the same line of treatment. The patient was shifted to the recovery unit for further management and close observation. The patient again presented with complaints of abdominal pain, dyspnea, unable to speak, and inability to pass stools. The patient was found to have tachycardia, tachypnea with hypotension, generalized body ache, guarding, and rigidity present on abdominal examination; hence, computed tomography (CT) of the abdomen was done, suggestive of mild pneumoperitoneum and mild ascites with associated diffuse peritoneal enhancement. This is noted secondary to a tiny rent suspected in the posterior wall of distal ileal loops in the region of the right paramedian pelvis (Fig. 2). Dilatation of small bowel loops with air-fluid level was also observed, with this dilatation identified up to the level of the terminal ileal loop. The transition point was identified as proximal to the ileocecal junction. No obvious mechanical lesion was seen at the transition point. Focal changes of pneumatosis intestinalis were noted in an ileal loop in the left lumbar region. Little air-containing outpouching was also observed in the proximal ileal loop in the left anterior paramedian infraumbilical abdominal cavity. Visualized lower chest reveals diffuse dense ground glassing with reticular abnormality, likely related to alveolar hemorrhage in this known case, which revealed perforation of indeterminate origin. The patient was taken for emergency exploratory laparotomy, in which ileal perforation was found. So closure of perforation with drainage of intra-abdominal collection was done. The postoperative condition remained stable. In view of fecal peritonitis, his steroid doses were reduced and titrated according to the clinical scenario. Biopsy and histopathological examination (HPE) findings were suggestive of fibrino-inflammation changes with serositis, with no evidence of malignancy. His condition gradually improved within a period and was discharged.
OD investigations | Day 1 | Day 7 | Day 15 | Day 28 | Day 35 | Day 41 | Day 46 |
---|---|---|---|---|---|---|---|
Hb (g%) | 6.7 | 7.4 | 7.6 | 8.1 | 10.9 | 9.1 | 9.5 |
WBC | 25,930 | 13,300 | 14,580 | 12,470 | 13,550 | 9,920 | 8,110 |
Platelet | 6,00,000 | 1,78,000 | 1,79,000 | 1,88,000 | 13,550 | 2,26,000 | 3,79,000 |
Na+ (meq/L) | 139 | 144 | 142 | 132 | 134 | 140 | 142 |
K+ (meq/L) | 5.0 | 4.4 | 4.9 | 4.2 | 3.8 | 3.1 | 3.3 |
Serum creatinine (mg%) | 1.64 | 1.65 | 1.38 | 1.14 | 1.30 | 0.81 | 0.8 |
CRP (mg/dL) | 151.6 | 28.4 | 242.9 | 15.1 | 28.5 |
CRP: C-reactive protein; Hb: hemoglobin; WBC: white blood cell
Fig. 1: Chest X-ray with DAH
Fig. 2: Computed tomography of the abdomen
DISCUSSION
The case highlights the atypical manifestation of ANCA-associated vasculitis with simultaneous occurrence of DAH and ileal perforation. DAH is one of the most common presentations of complicated ANCA vasculitis.1 The incidence of DAH in ANCA-associated vasculitis is between 8 and 36%, as per Arulkumaran et al. who suggest the disease is not so common.2 Along with the disease-related complication DAH, mortality is also high, around 50%.3,4 This presentation poses a diagnostic challenge as these complications are not commonly associated with the disease. Clinicians should be aware of the potential for such rare manifestations and consider them in the differential diagnosis. The pathophysiological mechanisms underlying the development of DAH and ileal perforation in ANCA-associated vasculitis remain unclear. It is hypothesized that the inflammatory process in small blood vessels leads to vascular damage and subsequent bleeding in the lungs. The management of DAH and ileal perforation in ANCA-associated vasculitis requires prompt and aggressive treatment to control underlying vasculitis and manage the complications. Likewise, Tripathi et al.,5 as in our case report, we treated our patient with methylprednisolone and rituximab, unlike plasma exchange. As in our case report, Thickett et al. suggest continuing with immunosuppressive therapies like methylprednisolone and rituximab.6 Akbulut7 and Sulaiman et al.’s8 case report also found intestinal perforation, like in our case report, with ANCA vasculitis. Ko et al.9 also support the treatment protocol as we used rituximab in the treatment of our patient as well. The case report outlines the therapeutic interventions employed in this particular case, including immunosuppressive therapy, corticosteroids, and supportive care. It emphasizes the need for close monitoring of the patient’s respiratory and gastrointestinal status and the importance of early surgical intervention in cases of perforation. Further studies are required for the evaluation of treatment protocols.
CONCLUSION
This case underscores the importance of considering rare and atypical presentations of ANCA vasculitis. Clinicians should maintain a high index of suspicion for potential multi-organ involvement, as timely recognition and appropriate management are crucial for optimal outcomes. Further research is needed to elucidate the pathogenic mechanisms linking ANCA vasculitis, DAH, and ileal perforation along with the management of the same.
ORCID
Bhavin Patel https://orcid.org/0000-0001-5070-9315
Ripal Shah https://orcid.org/0000-0003-4327-8304
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