11.16.15 VA Ambulatory Report Pearls – Pericarditis + Autoimmune Disorders and a word on AIP

We discussed the overlap between pericarditis/pericardial involvement and systemic autoimmune diseases. Below is a list of autoimmune disease, the type of pericardial involvement, and the estimated frequency of involvement (stolen from UpToDate).

Systemic Disease Frequency of pericardial involvement Type of pericardial involvement
Connective Tissue Diseases
Systemic lupus erythematosus > 50 Pericarditis, pericardial effusion
Rheumatoid arthritis 10-30 Pericarditis (10), pericardial effusion (30)
Systemic sclerosis 20-60 Pericarditis, pericardial effusion
Polymyositis and dermatomyositis < 10 Pericarditis, pericardial effusion, cardiac tamponade
Mixed connective tissue disease 10-30 Pericarditis, pericardial effusion
Sjogren syndrome < 30 Pericarditis
Behcet’s disease Rare Pericarditis
Granulomatous Diseases
Sarcoidosis Rare Pericarditis, pericardial effusion
Autoinflammatory Diseases
Familial Mediterranean Fever 1 Pericarditis
TRAPS Rare Pericarditis

Finally, we discussed a possible diagnosis of acute intermittent porphyria (AIP). Here a couple of learning morsels:

  • Autosomal dominant
  • Symptoms, which are usually triggered by a variety of factors – most commonly medications – are often vague and nonspecific making the diagnosis of AIP difficult
  • Two most common categories of presenting symptoms are gastrointestinal and neurologic in nature and symptoms typically resolve between attacks though patients can develop chronic symptoms
    • Abdominal pain = most common, earliest symptom in AIP
      • Occurs in 85-95% of patients with acute attacks
      • Severe, steady, and poorly localized
      • Commonly present with constipation, bloating, nausea, vomiting, and signs of ileus with abdominal distension and decreased bowel sounds
    • Peripheral neuropathy
    • Effects on the autonomic nervous system – tachycardia (80% of attacks), hypertension, sweating, restlessness, and tremor
    • Insomnia
    • Neuropsychiatric manifestations – anxiety, restlessness, agitation, hallucinations, hysteria, disorientation, delirium, depression, phobias, and altered consciousness
    • Neuropathic bladder dysfunction – dysuria, hesitancy, retention, and incontinence
      • Dark or reddish-brown urine is often an early symptom
  • Diagnosis = spot urine for porphobilinogen, porphyrins, and creatinin

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