Lofgren Syndrome: acute presentation with fever, bilateral hilar adenopathy, erythema nodosum or ankle arthralgia
Incidental finding in chest imaging: >90% of patients have pulmonary or thoracic lymphadenopathy on presentation and ~50% of patients present with only incidental radiological findings
Evaluation
Combination of clinical features, radiographic manifestations, exclusion of other similarly presenting diseases, and noncaseating granulomas on pathology
CXR: hilar and mediastinal lymphadenopathy ± pulmonary infiltrates. CXR stages are defined as below (stages do not represent disease activity)
Stage 3: pulmonary infiltrates without hilar adenopathy
Stage 4: pulmonary fibrosis that mainly involves upper lung zones
High-resolution chest CT: lymphadenopathy (bilateral and symmetric), perilymphatic micro or macronodules, fibrotic changes (reticular opacities, traction bronchiectasis, volume loss, cysts)
PFTs: may show restrictive disease (decreased TLC & VC) and diffusion impairment (reduced DLCO). Occasionally obstructive with endobronchial disease.
Labs: CBC w/ diff, CMP, UA, quant-gold for TB or tuberculin skin test, HIV. Depending on endemic fungi, serologic testing for histoplasmosis or coccidiomycosis can also be included.
ECG: should be obtained since AV block is the most common finding of cardiac sarcoidosis.
Biopsy
Important to rule out mimics. The differential for “noncaseating granulomas” is extensive, including lymphoma and fungal infections
Not required for patients with asymptomatic bilateral hilar adenopathy or pathognomonic presentations including Lofgren syndrome and some cases of lupus pernio
Management
Most do not require therapy: monitor symptoms, CXR, PFTs at 3-6 month intervals
Indications for treatment: highly symptomatic, progressive disease or severe disease at presentation
Mainstay of treatment is oral steroids
Dosing usually 0.3-0.6 mg/kg daily for 4-6 weeks
If only symptom is cough, could consider inhaled glucocorticoids
If unresponsive or unable to tolerate steroids may require alternative agents (MTX, AZA, TNFi)
Fibromyalgia
Tina Arkee
Background
Heterogenous chronic pain syndrome characterized by widespread, constant pain and fatigue
Incidence: 2-3% of adults in the United States, and up to 5% of adults worldwide. Similar prevalence in men and women but discordant diagnosis (account for more than 80% of diagnosed cases). Most often onset at 30-50y, but can occur at any age
Etiology: poorly understood but likely multifactorial in the setting of hypersensitization to pain, physiologic and emotional/psychological stressors, sleep disturbances, and genetic and environmental factors
Presentation
Commonly presents with widespread musculoskeletal pain, fatigue, and sleep disturbances lasting at least 3 months and NOT explained by another medical condition
Patients may report concomitant brain fog, depression/anxiety, or GI symptoms RHEUMATOLOGY 604
Physical exam is most notable for tenderness to palpation of multiple sites, hyperalgesia (greater pain than expected), and allodynia (pain to nonpainful stimuli)
Evaluation
Rule out other medical conditions with a thorough history, exam, and labs and imaging as indicated (with the caveat that patients can have fibromyalgia and comorbid conditions)
Workup: consider TSH, CK, CRP/ESR, vitamin D, CBC, CMP; additional workup based on presentation
In unclear cases, can use the 2016 ACR fibromyalgia diagnostic criteria to assist: (1) generalized pain in at least 4 of 5 body regions (2) that is present for at least 3 months and (3) not attributed to another medical condition, AND (4): WPI of 7+ and SSS of 5+ OR WPI of 4-6 and SSS of 9+ (see below)
Monitor symptom evolution with pain using patient-reported outcome scales:
Widespread Pain Index (WPI, 0-19): areas on the body where a patient experienced pain in the past week
Symptom Severity Score (SSS, 0-12): severity of symptoms including fatigue, unrefreshing sleep, cognition, headache, abdominal pain or cramps, and depression
Management
1st line; Multidisciplinary approach with initial focus on non-pharmacologic measures such as low-impact exercise (can be very beneficial), optimizing sleep hygiene, ruling out sleep disorders, mindfulness and meditation, cognitive-behavioral therapy, and treating any mood disorders
2nd line: for refractory cases, start medications based on the predominant symptom (pain, mood, or sleep)
FDA-approved: Pregabalin (pain and sleep), duloxetine (pain and depression/anxiety), and milnacipran (pain and mood)
Off-label: TCAs (low-dose amitriptyline or nortriptyline for sleep and/or pain), gabapentin, SSRIs, muscle relaxers, low-dose naltrexone
