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 (do not biopsy EN – it looks the same regardless of cause).
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
Many patients with isolated pulmonary disease may 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, vitamin C, CBC, CMP, sleep study; 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
