Autoimmune rheumatic diseases (AIRDs) are known for their systemic presentations and multi-organ involvement. Numerous infectious diseases, particularly mycobacterial, fungal and indolent bacterial infections endemic to specific geographic regions, present with varied signs and symptoms of multi-system involvement and can mimic AIRDs. Thus, differentiating infection from an AIRD is critical to resolve competing treatment approaches. This…
Renal osteodystrophy is associated with chronic kidney disease (CKD) and its associated metabolic derangements, most commonly CKD stages 3–5. It is often subclassified into four histological subtypes, with definite distinctions unable to be made clinically. These four subtypes, which may only be differentiated by bone biopsy, include: osteitis fibrosa cystica, mixed uremic osteodystrophy, osteomalacia and…
“Our patients’ goals for their care aren’t always the same as ours. We’re typically focused on disease control, and they’re often focused on quality of life. Having ongoing discussions about care goals is the only way to know when it’s time to switch gears. Would you know when to refer your patients to palliative care?…
Granulomatosis with polyangiitis (GPA) is a small vessel vasculitis associated with anti-neutrophil cytoplasmic antibodies (C-ANCA), specifically proteinase 3 (PR3) ANCA. GPA classically presents with multi-organ involvement. The first description of GPA defined a triad of features: 1) systemic necrotizing angiitis, 2) necrotizing inflammation of the respiratory tract and 3) necrotizing glomerulonephritis.1 Few cases of digital…
Shrinking lung syndrome (SLS) is a rare cause of dyspnea that has been most commonly described in patients with systemic lupus erythematosus (SLE), but is also found in systemic sclerosis, Sjögren’s disease and rheumatoid arthritis. Shrinking lung syndrome is characterized by a restrictive pattern on lung spirometry, despite normal lung parenchyma, and an elevated diaphragm.1…
Kidney involvement is a major cause of morbidity and mortality in systemic lupus erythematosus (SLE). Collectively termed lupus nephritis, SLE with kidney involvement comes in many subtypes. The current classification by the International Society of Nephrology/Renal Pathology Society (ISN/RPS), however, does not include lupus podocytopathy, which, through various clinical and epidemiologic studies, has recently been…
Adult-onset Still’s disease (AOSD) is a systemic autoinflammatory disorder characterized by persistent fever at regular intervals, arthralgias or arthritis, rash, sore throat and neutrophilic leukocytosis.1,2 Significant elevation in ferritin levels is characteristic and tends to correlate with disease activity. Additional clinical features may include myalgias, lymphadenopathy, hepatosplenomegaly, serositis, myocarditis, abnormal liver function tests and development…
Pachydermoperiostosis (PDP), also known as Touraine-Solente-Golé syndrome or primary hypertrophic osteoarthropathy, is a rare syndrome that can be inherited as autosomal dominant, autosomal recessive, or sporadically. This progressive disease primarily affects males, who tend to have more severe features than females. PDP usually occurs during adolescence, often starting around puberty.1 The main clinical features are…
The Case A 47-year-old woman presented with a one-year history of bilateral submandibular gland swelling, mild symptoms of xerostomia and xerophthalmia and arthralgias in her fingers. A review of systems was otherwise unremarkable. On physical examination, her submandibular glands on both sides were enlarged and had a firm texture. Her parotid glands were normal, as…
Pneumatosis cystoides intestinalis (PCI) is a rare gastrointestinal pathological process defined by the presence of gas within the layers of the intestinal wall, commonly within the mucosa and submucosa of the small and large intestines.2,3 PCI has been described in the literature in association with various connective tissue diseases, including scleroderma, mixed connective tissue disease,…