Sarcoidosis

Hypersensitivity pneumonitis is a mixed type III/IV immune reaction to repeated inhalation of an organic antigen in a sensitized host, producing noncaseating granulomas and bronchiolocentric inflammation. Removal of the offending antigen is the essential first step in treatment; glucocorticoids are added for significant hypoxemia or symptom burden, and unremitting exposure can progress to fibrotic lung disease indistinguishable from idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis is a chronic, progressive fibrosing lung disease of unknown cause defined by a usual interstitial pneumonia (UIP) pattern, presenting with progressive dyspnea, dry cough, bibasilar Velcro crackles, and clubbing in older smokers. A definite UIP pattern on high-resolution CT confirms the diagnosis without biopsy; management centers on antifibrotic therapy (pirfenidone or nintedanib), oxygen, and early lung transplant referral, since corticosteroids are not indicated and median survival is 3 to 5 years.

Tuberculosis, caused by Mycobacterium tuberculosis, remains a leading global cause of infectious death and presents as latent infection, primary/secondary pulmonary disease, or extrapulmonary and disseminated (miliary) disease. Diagnosis rests on AFB smear, NAAT, and mycobacterial culture (the gold standard), and drug-susceptible active disease is treated with a four-drug regimen (isoniazid, rifampin, pyrazinamide, ethambutol) for 2 months followed by isoniazid plus rifampin for 4 to 7 months.

Histoplasma capsulatum is an endemic dimorphic fungus, acquired by inhalation of conidia in the Mississippi and Ohio River valleys and other endemic regions, that most often causes asymptomatic or mild pulmonary infection but can disseminate in immunosuppressed hosts; diagnosis rests on the Histoplasma urinary antigen assay and treatment is itraconazole for mild to moderate disease or liposomal amphotericin B followed by itraconazole for severe or disseminated infection.

Coccidioidomycosis, or valley fever, is a dimorphic fungal infection acquired by inhaling Coccidioides arthroconidia in the southwestern United States, ranging from self-limited pneumonia to disseminated or meningeal disease, diagnosed by culture, histopathology, or serology and treated with fluconazole for symptomatic disease and lifelong fluconazole for meningitis.

Hodgkin lymphoma is a B-cell malignancy defined by Reed-Sternberg cells that classically presents with painless, contiguously spreading cervical or mediastinal lymphadenopathy in a young adult, and it is confirmed by excisional lymph node biopsy. Ann Arbor/Lugano stage and interim PET response drive risk-adapted ABVD chemotherapy with or without radiation, and most patients are cured.

Adult-onset Still disease is a rare systemic autoinflammatory disorder marked by quotidian spiking fevers, an evanescent salmon-colored rash, arthritis, sore throat, and a markedly elevated ferritin. It is a diagnosis of exclusion confirmed by the Yamaguchi criteria, and treatment begins with NSAIDs, escalates to glucocorticoids, then to interleukin-1 blockade (anakinra or canakinumab) or interleukin-6 blockade (tocilizumab) for refractory disease.

Hypercalcemia is an elevated serum calcium concentration, most often caused by primary hyperparathyroidism in ambulatory patients or malignancy in hospitalized patients. Mild disease requires only treatment of the underlying cause, while severe or symptomatic hypercalcemia is treated with aggressive IV isotonic fluids, followed by an IV bisphosphonate or denosumab for sustained control and subcutaneous calcitonin as a rapid but short-lived bridge.

Sjogren syndrome is a systemic autoimmune exocrinopathy in which lymphocytic infiltration of the lacrimal and salivary glands causes keratoconjunctivitis sicca and xerostomia, occurring alone (primary) or alongside another rheumatologic disease such as rheumatoid arthritis or lupus (secondary). Diagnosis combines the anti-Ro (SSA) and anti-La (SSB) antibody panel, objective dryness testing, and labial salivary gland biopsy under the 2016 ACR/EULAR classification criteria. Management centers on sicca-directed therapy, pilocarpine or cevimeline for xerostomia and topical cyclosporine for keratoconjunctivitis, with hydroxychloroquine or immunosuppression for extraglandular disease.

Granulomatosis with polyangiitis (GPA) is an antineutrophil cytoplasmic antibody (ANCA)-associated necrotizing granulomatous vasculitis of the upper airway, lower airway, and kidney, most often with cytoplasmic ANCA (c-ANCA) targeting proteinase 3 (PR3). Diagnosis rests on the clinical triad, ANCA serology, and tissue biopsy showing pauci-immune vasculitis, and treatment begins with high-dose glucocorticoids plus rituximab or cyclophosphamide for organ-threatening disease, followed by rituximab or azathioprine maintenance.

Erythema nodosum is a delayed hypersensitivity panniculitis presenting as symmetric, tender, erythematous subcutaneous nodules on the anterior shins, most commonly following streptococcal pharyngitis but also linked to sarcoidosis, inflammatory bowel disease, Coccidioides, Yersinia, pregnancy, and drugs such as oral contraceptives. Diagnosis is clinical, and management is directed at the underlying cause with NSAIDs, bed rest, and cool compresses for symptom control.

Uveitis is inflammation of the uveal tract, classified by anatomic location as anterior, intermediate, posterior, or panuveitis and by systemic association such as HLA-B27 spondyloarthritis, sarcoidosis, or Behcet disease. Anterior uveitis presents with a painful red eye and photophobia diagnosed at the slit lamp, while posterior uveitis presents with painless floaters or vision loss, and treatment is topical corticosteroid for anterior disease escalating to systemic corticosteroid and steroid-sparing immunosuppression for recurrent, severe, or posterior-segment disease.

Pulmonary hypertension is a resting mean pulmonary artery pressure of 20 mmHg or greater on right heart catheterization, classified into five WHO groups by mechanism. Diagnosis requires screening echocardiography followed by confirmatory right heart catheterization, and management is group-specific, with PAH-targeted vasodilator therapy reserved for group 1 disease.

Sarcoid arthropathy is the joint, periarticular, and bone manifestation of sarcoidosis, presenting acutely as Lofgren syndrome (bilateral hilar lymphadenopathy, erythema nodosum, and ankle arthritis, 95 percent specific and biopsy-sparing) or as a chronic granulomatous polyarthritis. Acute disease is usually self-limited and treated with an NSAID, while persistent or chronic disease escalates to glucocorticoids, then methotrexate or a tumor necrosis factor inhibitor.