Granulomatosis with polyangiitis (GPA) is a type of small-sized blood vessel vasculitis that predominantly affects the upper airways, lungs and kidneys and associates with the presence of anti-neutrophil cytoplasmic antibodies (ANCA). Nevertheless, any organ of the body can be affected by GPA, including the eye. Occasionally, ocular involvement can be the initial manifestation, thus representing an essential clue for the physician in the early diagnosis of the disease.
We present the case of a 53-year-old woman in whom recurrent episcleritis was the first sign of a multisystem disease. All further investigations led to the final diagnosis of GPA. The remission induction therapy chosen by the rheumatologist consisted of intravenous cyclophosphamide (CP) and methylprednisolone pulse-therapy, followed by oral glucocorticoids (GC). Based on the favorable clinical and paraclinical evolution, induction therapy was replaced by remission maintenance therapy. Azathioprine (AZA) was initiated and oral GC were continued, with dose tapering. Complete remission of episcleritis was observed.
Abbreviations:
GPA = granulomatosis with polyangiitis, EGPA = eosinophilic granulomatosis with polyangiitis, MPA = Microscopic polyangiitis, ANCA = Anti-neutrophil cytoplasmic antibodies, c-ANCA = ANCA to proteinase-3, p-ANCA = ANCA to myeloperoxidase, ELISAs = antigen-specific enzyme-linked immunosorbent assays, ENT = ear, nose, throat, CP = cyclophosphamide, NSAIDs = nonsteroidal anti-inflammatory drugs, AZA = azathioprine, GC = glucocorticoids
Acute polyarthritis requires a comprehensive differential diagnosis in order to establish its etiology. Moreover, sometimes it may unfold unexpected comorbidities, thus necessitating an individualized management approach.
This article describes the case of an elderly patient whose initial presentation of acute polyarthritis was interpreted as crystal induced-arthritis demonstrated by the presence of calcium pyrophosphate and lipid crystals in the synovial fluid. The clinical deterioration of the patient along with the suspicion of a systemic infection required interdisciplinary collaboration and led to his transfer to the infectious diseases department where the diagnosis of sepsis from unknown origin was added. Further investigations unmasked a large complicated pancreatic pseudocyst that eventually ruptured into the peritoneal cavity and led to the patient's death.
Vasculitides are inflammatory disorders which affect the blood vessels. There are three major categories of vasculitides depending on the size of the injured vessels. Although each category can affect any size artery, usually large vessel vasculitis affects large arteries. Takayasu arteritis and Giant Cell arteritis are known to be the two main types of large vessel arteritis. We present the case of a patient with a delayed diagnosis of Takayasu disease who sequentially develops a series of complications. The atypical evolution of the disease confirms once again the complexity of the vasculitic disorders and raises questions about the understanding of their pathophysiologic mechanisms.
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