Isolated pauci-immune pulmonary capillaritis associated with an extensive bilateral hemothorax and pleuro-pericarditis
Italian Journal of Medicine
; 16(SUPPL 1):39, 2022.
Article
in English
| EMBASE | ID: covidwho-1913139
ABSTRACT
Introduction:
Isolated pauci-immune pulmonary capillaritis (IPIPC) is a rare disorder characterized by small vessel vasculitis limited to alveolar capillaries in the absence of systemic manifestations. There are very few case reports of this disorder in the medical literature. Case Report A 37-yo male with no known history of autoimmune pathology who was admitted to the hospital for evaluation and treatment of dyspnea and thoracalgia. Peripheral blood cultures, serum studies to detect Legionella and Pneumococcus antigens, and a nasopharyngeal swab test for covid-19 were all negative. Chest imaging revealed bilateral pleural effusions from the base to the apices with concomitant atelectasis of the adjacent lung parenchyma. Although the results of an 18F-PET-CT scan revealed no pathological uptake, video-assisted thoracoscopy revealed diffusely edematous pleura with crater-like patches with new onset of venous vessel varicosities, intra-alveolar hemorrhages associated with disordered vascularization, suggesting small vessel vasculitis. Histologic findings included widespread intra-alveolar hemorrhage with organizing injury, hemosiderin-laden macrophages, scattered intra-arterial thrombi, and diffuse perivascular neutrophilic infiltrates consistent with a diagnosis of capillaritis.Conclusions:
Given the negative immune studies (save for a weakly-positive lupus anticoagulant and no evidence for extra-pulmonary vasculitis, the diagnosis was Isolated pauci-immune pulmonary capillaritis. The patient recovered in response to immunosuppressive/anti-inflammatory therapy.
antigen; endogenous compound; lupus anticoagulant; adult; artery thrombosis; atelectasis; autoimmune disease; blood culture; capillaritis; capillary; case report; clinical article; conference abstract; COVID-19 testing; dyspnea; hematothorax; hemosiderin laden macrophage; histology; human; human cell; human tissue; injury; Legionella; lung alveolus; lung hemorrhage; lung parenchyma; male; medical literature; nasopharyngeal swab; neutrophil chemotaxis; nonhuman; pericarditis; pleura effusion; positron emission tomography-computed tomography; pulmonary vasculitis; small vessel vasculitis; Streptococcus pneumoniae; thoracoscopy; thorax; vascularization; vein; videorecording
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Collection:
Databases of international organizations
Database:
EMBASE
Language:
English
Journal:
Italian Journal of Medicine
Year:
2022
Document Type:
Article
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