Unclassified Vasculitis with Episcleritis, Thrombophlebitis, Deep Vein Thrombosis, Pulmonary Vasculitis, and Intracranial Vasculitis: An Autopsy Case Report

Abstract

Patient: Male, 67 Final Diagnosis: Unclassified vasculitis Symptoms: Fever • ocular pain • erythema • chest pain • headache Medication: — Clinical Procedure: Biopsy Specialty: Rheumatology Objective: Rare disease Background: Systemic vasculitides constitute heterogenous conditions affecting many organs and systems through blood vessel inflammation. Although there are some classifications for vasculitis, several vasculitides are “unclassified” because they cannot be clearly assigned to one of the known entities. Case Report: We report an autopsy case of a 67-year-old Japanese man who presented with fever, ocular pain, erythema, chest pain, and headache. The disease caused episcleritis, thrombophlebitis, extensive deep vein thrombosis, multiple pulmonary nodules and masses, hypertrophic pachymeningitis, and hyper-intensity areas in brain parenchyma on magnetic resonance images. Histopathology of the pulmonary nodule confirmed vasculitis affecting medium-to-small veins and arteries without necrotizing vasculitis or granulomatous inflammation. We diagnosed the patient with unclassified vasculitis based on the clinicopathological characteristics. Steroids in combination with immunosuppressants were used, but the disease was refractory and relapsing. The disease activity was eventually controlled with rituximab, but the patient died of bronchopneumonia. On autopsy, lung and brain findings indicated healed vascular inflammation. Conclusions: This is the first case report of unclassified vasculitis, which is characterized as medium-to-small-sized arteritis and phlebitis, causing episcleritis, thrombophlebitis, deep vein thrombosis, pulmonary vasculitis, and intracranial vasculitis. The clinical conditions share some similarities with granulomatosis with polyangiitis and Behçet’s disease; however, they meet no classification criteria of any specific vasculitis. More cases need to be analyzed to confirm our findings.

Multifocal Thrombophlebitis and Orbital Cellulitis

“…Our 2 cases of multifocal thrombophlebitis and orbital cellulitis resemble the case described recently by Watanabe et al [10].…”

“…Recently, we read with great interest the article from Watanabe et al, describing the case of episcleritis, thrombophlebitis, deep vein thrombosis, along with lung and central nervous system involvement, potentially representing a new vasculitic entity [10]. Herein we report 2 cases of multifocal superficial and deep thrombophlebitis, orbital cellulitis and episcleritis; cases to a degree similar to the one reported by Watanabe et al Taken together, these 3 cases might indicate a unique vasculitic syndrome.…”

Episcleritis; and posterior uveitis misdiagnosed as orbital cellulitis in a child patient with Behçet’s disease

“…11 Episcleritis can occur with rheumatoid arthritis, juvenile idiopathic arthritis, reactive arthritis, systemic lupus erythematosus, inflammatory bowel disease, vasculitis, leukemia, Herpes simplex and zoster, Yersina infections, Lyme disease, and tuberculosis. [12][13][14][15][16][17][18][19][20][21][22][23] We ruled out these causes in this patient. An important point to note in our study was misdiagnosing the patient with preseptal cellulitis due to the first manifestations of the patient which were swelling and redness around the left eye.…”