Acute Rheumatic Fever

Today, we talked about the very interesting case of a middle-aged man who presented with acute migrating oligoarthritis, found to be febrile with an inflammatory synovial fluid and elevated ASO titers consistent with acute rheumatic fever!


Clinical Pearls

  • Nonsuppurative manifestations of GAS infection include acute rheumatic fever (ARF), acute GN, and Scarlet fever.
  • Use the modified Jones Criteria to help you diagnose ARF and treat early if high suspicion for the disease (do not wait for titers to come back).
  • Late complications of ARF include rheumatic heart disease (10-20 years after infection) and Jaccoud arthropathy.
  • Treatment of ARF involves NSAIDs for arthritis, PCN G IM x 1 dose for acute presentation and then monthly for prophylaxis, and patient education about oral hygiene to prevent endocarditis and need for prophylaxis before invasive procedures.

Differential diagnosis for a migratory arthritis

  • Rheumatic fever
  • Infective endocarditis
  • Vasculitis (IgA, cryo, ANCA associated)
  • SLE 
  • Acute leukemia
  • Serum sickness
  • Viral arthritis
  • Bacteremia (staph, strep, mening/gonococcal)
  • Pulmonary infections (mycoplasma, histoplasma)
  • Lyme
  • Whipple’s

Nonsuppurative complications of GAS infection

  • ARF
  • Scarlet fever
  • Acute GN

Rheumatic fever 

  • Nonsuppurative sequela that occurs 2-4 weeks after GAS pharyngitis
  • Epi
    • More common in children 5-15 years of age
    • More common in resource limited settings
  • Pathogenesis:
    • Poorly understood, ?molecular mimicry
  • Clinical manifestations:
    • Two primary manifestations of disease

Two manifestations of ARF

(Table above from UpToDate)

  • Late sequelae
    • Rheumatic heart disease (10-20 years after infection), primary involves the mitral valve >aortic valve.
      • Leading cause of cardiovascular death in the first 5 decades of life in resource limited settings
    • Jaccoud arthropathy
  • Diagnosis:
    • Revised Jones criteria (joint and cardiac manifestations can only be counted once).
      • Major
        • Carditis and valvulitis (clinical or subclinical) – 50-70%
          • Usually pancarditis. Valvulitis especially of mitral and aortic valves, shown as regurg on echo.
          • Carey Coombs murmur: short mid-diastolic murmur heard loudest at the apex
        • Arthritis (migratory, involving large joints) – 35-66%, earliest symptom
          • Several joints affected in quick succession, each inflamed for a day or two to one week. Most common are knees, ankles, elbows, and wrists.
        • CNS involvement (Sydenham chorea) – 10-30%
        • Subcutaneous nodules – 0-10%
        • Erythema marginatum – <6% 
      • Minor
        • Arthralgia
        • Fever >38.5
        • Elevated acute phase reactants (ESR, CRP)
        • Prolonged PR interval on EKG
      • Diagnosis requires evidence of prior GAS infection plus:
        • 2 major OR
        • 1 major + 2 minor criteria OR
        • 3 minor criteria (only if patient has history of prior episode of ARF)
      • In a high prevalence setting, slightly modified criteria are used.
    • Labs:
      • Prior GAS infection through either
        • Throat culture
        • Positive rapid strep antigen test
        • Elevated or rising ASO titers
      • Treatment
        • Goals
          • Symptomatic relief of acute disease manifestations
            • Arthritis: NSAIDs
            • Carditis: if severe, heart failure treatments
          • Eradication of GAS
            • IM PCN G benzathine x 1
            • Contacts (throat culture test and treat if positive)
          • Ppx against future GAS infection to prevent progression of cardiac disease
            • PCN G IM once a month
            • For 5 years or until 21 years of age (whichever is longer)
            • If ARF with carditis and residual heart disease
              • 10 years or until 40 years, sometimes even lifelong
            • Education
              • Oral health
              • Ppx before any invasive procedures

Neurosyphilis? Wait… reactive arthritis!

Thanks to Tim for presenting the interesting case of a middle-aged man with h/o inadequately treated syphilis who presented with neck stiffness worse in the mornings, back pain, and blurry vision, admitted for presumed neurosyphilis.  Exam revealed inflammation of T2/T3 joints, L SI joint tenderness, and an inflamed R foot with dactylitis of the 3rd and 4th digits.  Further history revealed a recent gonorrhea/chlamydia for which he was treated and HLA B27 positivity consistent with reactive arthritis!  He was started on NSAIDs with significant improvement of symptoms.


Clinical Pearls:

  • Neurosyphilis is most commonly seen in HIV positive patients and can present at any time after infection.
  • Early neurosyphilis occurs within the first year after infection and involves the CNS, meninges, and vasculature
    • Neurosyphilis presents with posterior uveitis or pan-uveitis whereas reactive arthritis presents with anterior uveitis
  • Late neurosyphilis occurs >10 years after infection and involves the brain and spinal cord parenchyma
  • The four main spondyloarthropathies are ankylosing spondylitis, psoriatic arthritis, reactive arthritis, and IBD-related arthritis.
  • The genital pathogen most commonly associated with reactive arthritis is chlamydia trachomatis.
    • HLA B27 is positive in 30-50% of patients
    • Mainstay of treatment is NSAIDs
    • Disease typically lasts 3-5 months.

 

Syphilis

Clinical manifestations and treatment of different stages of syphilis

Neurosyphilis manifestations

  • Refer to this prior post
  • Early (w/n first year of infection)
    • CSF, meninges, vasculature
    • Symptomatic meningitis
    • Ocular syphilis (posterior uveitis, panuveitis)
    • Meningovascular syphilis
      • Arteritis of any sized vessel which can lead tostroke or spinal cord infarction
  • Late
    • Brain and spinal cord parenchyma
      • General paresis (10-25 years after initialinfection)
        • Progressive dementia
        • Psychiatric symptoms
      • Tabes dorsalis (>20 years after initialinfection)
        • CSF may be completely normal
        • Affects dorsal columns
        • Symptoms
          • Sensory ataxia
          • Argyll-Robertson pupil
          • Lancinating pains
  • Diagnosis
    • Non-treponemal tests (poor sensitivity but highspecificity)
      • VDRL
      • RPR
    • Treponemal tests
      • FTA-ABS
      • Syphilis EIA
    • In an HIV negative patient with suspectedneurosyphilis and a non-reactive CSF-VDRL, one can establish the diagnosis with
      • CSF lymphocytes >5 cells/microL
      • CSF protein concentration >45

Reactive Arthritis

  • Epimiology
    • Young adults, M:F equal
  • Typically follows GI or urogenital infections (several days to weeks after infection)
    • Chlamydia trachomatis (most common genital infection associated)
    • Yersinia
    • Salmonella
    • Shigella
    • Campylobacter
    • E coli
    • C diff
    • Chlamydia pneumoniae
  • Manifestations
    • Mono- or oligoarticular pattern of arthritis,often involving the lower extremities, sometimes associated with dactylitis and enthesitis
    • The triad of arthritis, urethritis, andconjunctivitis is only present in a subset of patients (formerly called Reiter’s syndrome)
    • Ocular manifestions: conjunctivitis, less frequently anterior uveitis, episcleritis, and keratitis.
    • Other: 
      • Skin: keratoderma blennorhagica, erythema nodosum
      • Circinate balanitis 
      • Nail changes resembling psoriatic arthritis
  • Lab
    • E/o of antecedent or concomitant infection
    • Elevated acute phase reactants
    • Positive HLA-B27 (present in 30-50% of patients)
    • Inflammatory synovitis
    • Imaging consistent with enthesitis or arthritis
  • Treatment
    1. Treat any ongoing concurrent infection
    2. NSAIDs (first line)
    3. Steroids (if refractory to NSAIDs)
    4. DMARDS (for chronic reactive arthritis)
    5. Anti-TNF (last resort)
  • Prognosis
    • Duration is typically 3-5 months
    • >6 months duration is considered chronic reactive arthritis
    • Most remit completely or have little active disease w/n 6-12 months after presentation
    • 15-20% may experience more chronic persistent arthritis

Mycoplasma Induced Rash & Mucositis (MIRM!) 10/24/2018

Ernest presented a case of a young woman, with no medical history, presenting with acute onset severe mucositis (eyes, mouth, urogenital) after a few days of viral prodrome and one day after taking azithromycin prescribed by her PCP. Her skin findings were almost non-existent and the bulk of her symptoms were isolated to the mucosa. Her presentation is consistent with a diagnosis of MIRM!


MIRM (Mycoplasma Induced Rash and Mucositis)

Epidemiology

  • 25% of patients with mycoplasma pneumoniae experience extra-pulm manifestations
  • Coined different terms, incomplete SJS, Fuchs Syndrome, MIRM
  • Mean age: Young (median 11-12 yo), male predominance.

Presentation

  • Universally will have some sort of prodrome: cough, malaise, fever preceding eruption of lesions by ~ 1 week.
  • Manifestations: variable, mucositis alone, prominent mucositis with sparse skin involvement. Skin involvement tends to be very rare and on the milder side, presenting as vesiculobullous, targetoid, papules, macules. Rarely morbilliform.
  • Majority of cases are severe mucositis alone.
  • Involvement: Oral (100%), ocular (92%), urogenital (78%)

Diagnosis

  • Clinical Dx
  • Mycoplasma IgM/IgG helps but their sensitivity and specificity are highly variable.

Management:

Supportive care (especially pain control, hydration/nutrition, infection prevention) plus treat the underlying cause (mycoplasma)!

  • Systemic corticosteroids (mixed data so generally not recommended first line)
  • IVIG (has been used in very severe cases))

Prognosis

  • Better than SJS/TEN, 81% will make a full recovery.
  • Blindness/residual visual impairment is possible but less common vs SJS/TEN

Key distinguishing features:

MIRM: Young, slight male preference, usually 7 days after infection, predominantly mucosal involvement, very little cutaneous involvement, better prognosis vs SJS/TEN.

SJS/TEN: Any age, female preference, usually 1-3 weeks after drug exposure, diffused skin involvement (Nikolsky sign) + mucosal involvement, more severe ocular manifestation.

Please refer to this review article for more background on this condition.

Diffused Alveolar Hemorrhage (DAH) AND Hemophagocytic Lymphohistiocytosis (HLH) 10/22/2018

Thank you Charles for presenting this really interesting case. A 18 year old woman with a history of asymptomatic thrombocytopenia who presents with several days of non-specific fever, chills, malaise, mild shortness of breath and she was found to have acute anemia, thrombocytopenia, elevated transaminitis, and patchy bilateral pulmonary infiltrates on CXR during initial presentation. She became acutely ill with submassive hemoptysis and went into respiratory failure in 24-48 hours. She was found to have DAH on BAL. Her autoimmune and infectious work up came back negative, but her ferritin  came back at 75776. Base on this and her constellation of symptoms, further work up revealed a 6/8 criteria for diagnosis of hemophagocytic lymphohistiocytosis!


DAH

Presentation

  • Dyspnea, cough fever, respiratory failure, acute anemia
  • Hemoptysis only in 2/3 of cases
  • Definition: Hemoptysis, diffuse alveolar infiltrates, acute anemia, and hypoxemic respiratory failure

Pathophysiology

  • Widespread damage to pulmonary small vessels, leading to blood within the alveoli eventually causing impaired gas exchange.
  • Causes: Autoimmune/connective tissue disease leading to pulmonary vasculitis (ANCA, anti-GBM), certain pulmonary infections, toxins, drug reactions, mitral stenosis in some cases
  • 3 distinct histologic subtypes that can give hints to underlying pathology
    • Most common: Pulmonary capillaritis: ANCA vasculitis, GPA, EPGA, pauci-immune, Goodpasture, HSP, SLE, RA, APLS, MCTD, Behcet, drug-induced, lung transplant rejection, etc.
      • Systemic vasculitis manifestation
    • Bland pulmonary hemorrhage: Coagulopathy, mitral stenosis, toxin/inhalation, SLE, drugs, Goodpasture
      • Anti-GBM, SLE, no inflammation or destruction of capillaries but RBC leakage
    • Diffuse alveolar damage: BM transplantation, radiation, ARDS, cytotoxic drugs, other causes

Diagnosis

  • CXR: Diffuse bilateral alveolar infiltrates, no pathognomonic findings
  • BAL: serial bloody aspirate with sequential sampling
  • DAH
  • CT: Non-specific GGO
  • Biopsy: Tissue biopsy of the lung is definitive in confirmation of DAH but underlying cause might not be revealed.

Management

  • Treat underlying cause
  • Respiratory support, most patients die from respiratory failure
  • High dose corticosteroids, i.e. methylprednisolone up to 500mg Q6H (up to 2g daily)
  • Other agents: Cyclophosphamide, azathioprine, MTX, mycophenolate, etanercept.
  • Plasmapheresis for Goodpasture or vasculitidies.
  • Key: Early identification and treatment

HLH

Epidemiology

  • Worldwide incidence is unknown, not enough data available, thought to be rare AND underrecognized but growing recognitive leads to higher incidence.
  • Familial types: more common to occur in pts < 18yo
  • Secondary HLH: any age

Pathophysiology

  • Uncontrolled hyperinflammatory response with dysregulated macrophage activity leading to excessive cytokine production
  • Primary: HLH due to an underlying genetic abnormality or without clear cause
    • Autosomal recessive familial HLH
    • Idiopathic
  • Secondary: Due to something else
    • Retrospective study at Mayo in 2014:
      • Infection (34%), most commonly EBV
      • Autoimmune (8%), Macrophage activation syndrome (MAS), most often associated with AOSD, systemic juvenile idiopathic arthritis, or SLE.
      • Malignancy (52%) NHL, HL, acute leukemia
      • Idiopathic/Immune deficiency/other (6%)

Presentation

  • Fever, splenomegaly, cytopenias are most common
  • + manifestation of the trigger
  • Complications: Infection, DIC, bleeding complications (reports of intracranial hemorrhage, GIB, DAH), end organ damage.

Diagnosis: Per the Histiocyte Society: 5/8 criteria for diagnosis. In case you cannot remember all 8, please refer here for the famous HLH Song by Dr. Eric Lau:

    1. Fever
    2. Splenomegaly
    3. Peripheral cytopenia (> 2 cell lines)
    4. Hypertriglyceridemia or Hypofibrinogenemia
    5. Elevated ferritin > 500 (> 10000 = 90% sensitive and 96% specific for HLH)
    6. Low NK cell activity
    7. Elevated soluble CD25 (soluble IL2-R)
    8. Hemophagocytosis in BM, spleen, or LN: Only seen in later course of the diseases and not required for the diagnosis, neither sensitive nor specific, can be seen in severe sepsis/critical illness)

Management

  • Like all things in medicine, treat the underlying cause
  • Current treatment is based on the HLH-94 study on pediatric population
    • Induction: 8 weeks dexamethasone and etoposide.
    • Maintenance: Cyclosporine, tacrolimus, dex pulses
    • If MAS: Steroids alone, usually responsive.
    • Hematopoietic stem cell transplant is refractory/relapsing.

For more information on HLH, please refer to this article by Dr. Schram and Dr. Berliner published in Blood (as in the journal) in 2015.

On rhabdo and myopathies – 10/9/18

Thanks to Cameron and Adam for presenting the case of a middle aged man with no significant PMH who presented with diffuse myalgias and chronic progressive proximal muscle weakness, found to have a CK >12k and EMG findings concerning for an inflammatory myopathy, awaiting muscle bx for diagnosis.


Clinical Pearls

  • Rhabdomyolysis literally means dissolution of skeletal muscle and has a broad differential outside of the typical traumatic or exertional processes associated with it see below).
  • The four main inflammatory myopathies are dermatomyositis, polymyositis, inclusion body myositis, and necrotizing autoimmune myositis.
  • Polymyositis is rare and a diagnosis of exclusion after the other three main inflammatory myopathies have been investigated.
  • Overall, the prognosis of inflammatory myopathies is good with appropriate treatment.  The exception is inclusion body myositis which is a progressive disorder without any effective therapy.
  • Pigment nephropathy can occur with rhabdo regardless of the underlying etiology especially in patients with CK >5000.  Aggressive IV hydration to lower CK levels is important to reduce the risk of kidney injury.

Rhabdomyolysis:

DDx:

  • Traumatic
    • Crush injuries, surgery, prolonged compression from immobility or coma
  • Non-traumatic
    • Exertional:
      • Normal muscle: strenuous exercise, heat stroke, seizures, hyperkinetic states
      • Abnormal muscle: metabolic myopathies, mitochondrial myopathies, malignant hyperthermia, NMS
    • Non-exertional
      • Alcoholism
      • Drugs and toxins: lipid-lowering drugs (fibrates, statins), alcohol, heroin, cocaine, meth, colchicine
      • Infections: influenza, coxsackie, EBV, HIV, legionella
      • Electrolyte abnormalities: hypokalemia, hypophosphatemia, hypocalcemia
      • Endocrinopathies: DKA, HHS, hypothyroidism, vitamin D deficiency
      • Inflammatory myopathies (rare)
      • Paraneoplastic
      • Miscellaneous

Inflammatory myopathies

Largest group of potentially treatable myopathies in children and adults.

  • Four subtypes: distinguishing which process is important because each subtype has a different prognosis and response to therapy
    • DM
      • Anti-Mi-2, anti-MDA-5, anti-TIF-1, anti-NXP-2
    • PM
      • Rare, often misdiagnosed
      • Dx of exclusion
    • Necrotizing autoimmune myositis
      • More common than PM
      • Occurs alone or after viral infections or in association with cancer, CTD, or post-statin
      • Anti-SRP or anti-HMGCR
      • Highest CK level
    • Inclusion body myositis
      • Most common in people >50
      • 7.9 cases/million in the US
      • Distal muscles impacted first
      • Facial muscles impacted
      • Muscle atrophy occurs earlier than in others
      • Extramuscular manifestations are uncommon
      • Dysphagia occurs in >50%
      • Muscle atrophy is common
      • Lowest CK level
  • Up to 30% of patients with DM or PM have a constellation of clinical findings termed “antisynthetase syndrome”
    • Acute disease onset
    • Constitutional symptoms (fever, weight loss)
    • Myositis
    • Raynaud’s
    • Mechanic’s hands
    • Non-erosive arthritis
    • ILD
    • Labs show antibodies to tRNA synthetase enzymes (anti-Jo-1)
  • Extramuscular manifestations
    • systemic symptoms
    • cardiac arrhythmias or ventricular dysfunction
    • pulmonary complications (ILD)

Capture

Table above adapted from this and this review article by NEJM.

GPA – 10/1/18

Yours truly presented a case of a middle-aged woman with a recent history of otitis, sore throat, conjunctivitis, photophobia, and arthralgias who presented with chronic and progressive decline in functional status and AMS, found to be uremic with work up revealing c-ANCA associated ESRD.


Clinical Pearls

  • Remember that oval fat bodies are specific for glomerular pathology (more commonly nephrotic syndrome but can be seen in nephritic disease as well).
  • ANCA-associated vasculitides include GPA, MPA, eGPA (and renal-limited vasculitis).
  • All have similar features on renal histology (focal necrotizing, crescentic, pauci-immune glomerulonephritis).
  • They can affect multiple organ systems (see breakdown below) which makes their clinical diagnosis challenging apart from the following differences:
    • c-ANCA is associated with GPA, p-ANCA is seen in MPA and eGPA
    • Granulomas are seen in GPA and eGPA
    • Eosinophilia and asthma are associated with eGPA

ANCA-associated vasculitides

Capture

Chart above adapted from this paper by Koldingsnes et al.

Granulomatosis with polyangiitis (GPA)

Diagnostic criteria (two or more has 88% sensitivity and 92% specificity):

  • Nasal or oral inflammation (painful/painless oral ulcers, or purulent or bloody nasal discharge)
  • Abnormal chest radiograph showing nodules, fixed infiltrates, or cavities
  • Abnormal urinary sediment (microscopic hematuria w/w/o red cell casts)
  • Granulomatous inflammation on bx of artery or perivascular area

Clinical presentation:

  • Most commonly in older adults, M=F
  • More common among white individuals (~89%)
  • S/s
    • Fatigue, fever, weight loss, arthralgias, rhinosinusitis, cough, dyspnea, urinary abnormalities, purpura, and neurologic dysfunction.
    • ENT
      • 90% of GPA cases, only 35% of MPA
      • Nasal crusting, sinusitis, otitis media, earache, polychondritis, ulcers, discharge
      • Conductive and/or sensorineural hearing loss
      • Saddle nose deformity
    • Tracheal and pulmonary disease
      • Airways or parenchyma
    • Renal
      • ~18% at presentation but subsequently develops in 77-85% of patients within the first 2 years of disease onset
      • High risk of progression to ESRD
      • Asymptomatic hematuria
      • Subnephrotic range proteinuria
      • Rapidly progressive GN
    • Cutaneous
      • ~50% of patients
      • Leukocytoclastic angiitis is most common which causes purpura of lower extremities
      • Other findings: urticarial, livedo reticularis, nodules, erythema nodosum, pyoderma gangrenosum, and Sweet syndrome
    • Ophthalmic/orbital
      • Conjunctivitis, corneal ulcers, episcleritis/scleritis, optic neuropathy, retinal vasculitis, and uveitis.
    • Other organs
      • CNS: neuropathy, CN abnormalities, mass lesions, hearing loss, granulomatous inflammation of the CNS
      • GI tract, heart, lower GU, parotids, thyroid, liver, or breast
      • High incidence of DVT (unclear mechanism)
    • Can progress slowly over months or explosively over days
    • Relapses can manifest differently than original presentation

Diagnosis requires biopsy!

Treatment:

  • Prompt initiation of therapy can be life and organ sparing
  • Induction therapy: Steroids +-Cyclophosphamide +-Rituximab
  • Maintenance therapy: multiple options-Azathioprine, MTX, Rituximab, Leflunomide

Still’s Disease – 9/10/18

Thanks to Becky Lee yet again for presenting an interesting case of a young woman presenting with acute onset of fever and polyarthritis, found to have a history of similar episodes in the past together with a rash concerning for Still’s disease!


Clinical Pearls

  • Still’s disease is a diagnosis of exclusion!  Yamaguchi criteria can help with ruling in the diagnosis.
  • Still’s remains a multi-systemic disorder of unknown etiology because it’s difficult to diagnose and rare (0.16 cases per 100,000).
  • RF and ANA are generally negative but can be positive in <10% of patients with Still’s in low titers.
  • ~66% of patients present with sore throat secondary to cricothyroid perichondritis or aseptic nonexudative pharyngitis.
  • The disease is often recurrent.  Predictors of poor outcome include erosive polyarthritis on presentation and shoulder/hip involvement.

Acute polyarthritis (>5 joints involved):

Remember that for rheumatologic disorders, timing, symmetry, and number of joints involved is crucial to coming up with a differential diagnosis.  So for our patient with acute polyarthritis, consider the following:

  • Infection
    • Viral: hepatitis, HIV, parvovirus B19
    • Spontaneous bacterial endocarditis
  • Rheumatologic:
    • Rheumatoid arthritis
    • Reactive arthritis
    • SLE
    • Dermatomyositis
  • Vasculitis
    • PAN
  • Drug reactions
  • Auto-inflammatory or disease of the innate immune system (as opposed to autoimmune or diseases of the adaptive immune system):
    • Periodic fever syndromes (TRAPS, PFAPA, hyper-IgD syndrome)
    • Still’s disease
  • Schnitzler’s syndrome – chronic urticaria associated with a monoclonal gammopathy (usually IgM kappa)
  • Sweet syndrome – painful skin lesions
  • Sarcoid
  • Kikuchi disease – cervical LAD and fever (necrotizing lymphadenitis)
  • HLH/Macrophage activation syndrome – leukopenia and thrombocytopenia, elevated triglycerides, low fibrinogen and haptoglobin

Still’s disease:

  • Some clarifications on nomenclature:
    • Systemic juvenile idiopathic arthritis (sJIA): first presentation <17 years old, previously referred to as Still’s disease
    • Adult onset Still’s disease (AOSD): first presentation  > 17 years old
  • Epidemiology of AOSD:
    • 0.16 cases per 100,000
    • No sex predominance (F=M)
    • Bimodal age distribution with peak between 15-25 and another 36-46 years of age.  New diagnosis in patients >60 have been reported.
  • Clinical features ⇒ Yamaguchi criteria (need 5 total with  > 2 major)
    • Major criteria:
      • Daily fevers to 39
      • Arthritis >2 weeks
      • Non-pruritic salmon-colored macular/maculopapular rash on trunk or extremities (though cases of pruritic rash have also been reported)
      • ↑ WBC >10k, >80% neutrophils
    • Minor
      • Sore throat
      • LAD and/or splenomegaly
      • ↑ AST, ALT, or LDH
      • Negative ANA/RF
  • Treatment
    • Mild: NSAIDs
    • Moderate: NSAIDs + DMARDs
    • Severe: NSAIDs + DMARDs (IL1 receptor antagonists like anakinra appear to be more helpful than TNF inhibitors especially in sJIA)
  • Prognosis:
    • Overall good prognosis
    • Disease can be limited to one episode or recurrent over time
    • Poor prognostic indicators:
      • Hip and shoulder involvement
      • Erosive polyarthritis at initial diagnosis
  • Complications
    • Macrophage activation syndrome (ie HLH) can occur in 15% of cases
    • DIC
    • TTP
    • Diffuse alveolar hemorrhage
    • Pulmonary HTN
    • Aseptic meningitis

References: 

Great recent review article on Still’s disease (AOSD Review) and this prior post on our blog!