Retroperitoneal fibrosis (RPF) is a rare condition characterized by aberrant fibroinflammatory tissue developing in the retroperitoneum. This disorder was initially called Ormond’s disease. RPF may be idiopathic or secondary to other conditions. Idiopathic RPF is a part of the disease spectrum of chronic periaortitis due to its typical periaortoiliac localization. Idiopathic retroperitoneal fibrosis is a…
Intra-articular venous malformations of the knee are an uncommon cause of knee pain in children. Presenting symptoms of this rare subtype of venous malformation often include nonspecific pain and joint swelling, which may be episodic, and accompanied by limited range of motion. The signs and symptoms can mimic juvenile idiopathic arthritis (JIA). Timely diagnosis of…
Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis that typically affects medium-sized muscular arteries. The clinical subsets of PAN are idiopathic, generalized, secondary hepatitis B virus (HBV) associated and cutaneous PAN. These clinical subsets are important because of their therapeutic implications. Virtually any organ system can be affected in generalized PAN, but this vasculitis tends…
Granulomatosis with polyangiitis (GPA), is a type of anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) that affects small- to medium-sized vessels.1 It can occur equally in both men and women, with a reported mean age at onset of 55 years.2 The classical presentation of GPA includes upper respiratory tract (i.e., chronic sinusitis, serous otitis and…
Giant cell arteritis (GCA) is a granulomatous vasculitis of large- and medium-sized arteries, usually affecting the cranial branches of the aortic arch. It is the most common vasculitis, with the highest risk factor being age. Accurate diagnosis and prompt initiation of therapy are of great importance to prevent serious complications, with the most feared being…
The difference between Castleman disease and Castleman-like disease may be subtle, but it comes with significant ramifications. Case Presentation This case involves a pregnant 19-year-old woman who presents over multiple hospitalizations with concerns for systemic lupus erythematosus and macrophage activation syndrome. At 36 weeks’ gestation, the patient’s weight had dropped from 215 lbs. to 170…
Pulmonary manifestations in patients with systemic lupus erythematosus (SLE) include pleuritis, acute pneumonitis, interstitial lung disease, pulmonary arterial hypertension, shrinking lung syndrome and diffuse alveolar hemorrhage (DAH). DAH is a rare, but devastating, complication of SLE, with high mortality rates. The incidence of DAH in SLE ranges from 0.6% to 5.4%, but the mortality rate…
In September 2019, a previously healthy, 9-year-old white girl presented to the emergency department following two months of sinusitis and unexplained fever responsive to ibuprofen. She presented with anorexia; a 9 lb. weight loss; intermittent, nonbilious, nonbloody emesis; and occasional epistaxis with digital manipulation of the nose. Six weeks prior to admission, she had presented to…
Gout is the most common type of inflammatory arthritis in adults, and it typically occurs in men over the age of 50. When gout presents in younger patients or in women, this should warrant consideration of secondary causes. We describe an unusual genetic cause of tophaceous gout in a young, premenopausal woman. Case Report In…
Immunoglobulin G4-related disease (IgG4-related disease) is relatively rare, although with greater recognition it is becoming more frequently diagnosed. Some of the more common presentations of the disease include aortitis, cholangitis, autoimmune pancreatitis, sclerosing sialadenitis, orbital disease with proptosis and subacute mass development in the affected organ.1 It has protean manifestations and is diagnosed by classic…