An Interesting Case of Euthyroid Graves' Ophthalmopathy, With Negative Thyroid-Stimulating Hormone Receptor Antibodies.

Thyroid eye disease (TED), also known as Graves' orbitopathy or ophthalmopathy (GO) or Graves' eye disease, is an autoimmune condition of the retroocular tissues associated with Graves' disease. In isolated GO, the patient can present without thyroid hormone dysfunction or systemic symptoms of Graves' disease, in which case it is called euthyroid Graves' ophthalmopathy (EGO). It is very rare for this condition to have negative thyroid-stimulating hormone receptor (TSHR) autoantibodies, and we present such a rare case of a young female, who had progressive bilateral vision loss, intermittent left-sided retroocular headache, and severe bilateral proptosis. The patient was diagnosed with EGO based on multidisciplinary consults, diagnostic orbital magnetic resonance imaging (MRI) results, and a good response to treatment with intravenous steroids. Later, the patient was followed as an outpatient and treated with thyroid orbitopathy-specific immunotherapy with teprotumumab. The patient's response to teprotumumab was excellent and caused significant improvement in visual acuity, proptosis, and chemosis. This adds valuable literature to the medical field and gives insight to clinicians to consider the diagnosis of GO even with seronegative TSHR autoantibodies and euthyroid hormone status. It also adds to the understanding of the complex pathophysiology of this rare condition.

An Interesting Case of Immunoglobulin G4-Related Retroperitoneal Fibrosis Treated With Rituximab.

Immunoglobulin G4-related disease (IgG4-RD) is a fibroinflammatory condition. Its common manifestations include type I autoimmune pancreatitis and retroperitoneal fibrosis (RPF). We present a rare case involving a 43-year-old female who presented with left lower quadrant (LLQ) pain. Imaging of the abdomen and pelvis revealed left hydroureteronephrosis to the level of an inflammatory process in the left adnexal region, possibly reflecting a tubo-ovarian abscess (TOA). The gynecologic evaluation concluded that the mass was unlikely of gynecologic sources. Transgluteal biopsy of the mass was highly suggestive of IgG4-RD. The patient received prednisone and rituximab (RTX), resulting in complete resolution of the mass, which was confirmed on repeat imaging. This case report provides a valuable addition to the literature to highlight that the diagnosis of IgG4-RD is based on the combination of characteristic clinical, serologic, radiologic, and histopathologic findings. Also, it underlines that the management of the disease is through glucocorticoids (GCs) as the first-line agent for remission induction in all patients with active, untreated IgG4-RD. RTX therapy is an effective treatment for IgG4-RD that is refractory to GCs. Recent studies have suggested that RTX monotherapy can be used to induce and maintain remission in patients with IgG4-RD.

Purpura Fulminans: a Rare but Fierce Presentation of Pneumococcal Sepsis.

Infectious purpura fulminans (PF) is a rare presentation of disseminated intravascular coagulopathy (DIC) due to diffuse intravascular thrombosis and haemorrhagic infarction of the skin. PF can present in infancy/childhood or adulthood and usually presents as ecchymotic skin lesions, fever and hypotension. It is most commonly a consequence of sepsis related to Neisseria meningitidis, Streptococcus pneumoniae or Haemophilus influenzae. Despite aggressive management of sepsis with intravenous fluids, antibiotics, and conventional and nonconventional therapies, the condition still carries a mortality rate of 43%[1]. Streptococcus pneumoniae mostly presents with community-acquired pneumonia. We present a case of PF secondary to DIC related to Pneumococcal sepsis in an otherwise healthy and immunocompetent patient. LEARNING POINTS: Infectious purpura fulminans is a haematological emergency that demands early recognition and timely institution of therapy to prevent significant morbidity and mortality.A characteristic skin rash is a key diagnostic clue pointing to purpura fulminans, and should lead to prompt institution of therapy, as waiting for a skin biopsy result can delay the diagnosis and result in significant morbidity and mortality.Due to the lack of prospective data on management of the condition, various modalities, such as hyperbaric oxygen therapy and IVIG, still have questionable benefits. We therefore aim to expand knowledge of purpura fulminans management.

Giant Right Coronary Artery Aneurysm: A Case Report.

Coronary artery aneurysms are not very uncommon but 'giant' coronary artery aneurysms are rare, with a reported prevalence of 0.02% to 0.2%. Coronary artery aneurysm may be symptomatic or asymptomatic depending on their size and location but it is very unusual for a giant coronary artery aneurysm to be asymptomatic. Here, we present a case in which the giant coronary artery aneurysm remained undiagnosed and asymptomatic for several years.