A pt comes in with purpura, petechiae and hemorrhagic bullae in their mouth. What do they have?
idiopathic thrombocytopenia purpura (ITP); chief complaint - mucosal or skin bleeding
What Ig is involved with ITP? Which cell lines are affected?
autoimmune do where IgG antibody formed that binds to platelets; destruction in spleen; isolated thrombocytopenia (other cell lines normal)
For ITP you are asymptomatic till you start bleeding at...? What is your prognosis?
nose, soft palate, petechiae, gums, GI and CNS; good
What is the hallmark in lab findings for ITP?
thrombocytopenia with platelet counts less than 10,000/mcL (counts less than 5000/mcL can lead to CNS bleeding); peripheral smear, bone marrow and coagulation studies are normal
What is in the differential diagnosis for ITP?
hematologic malignancies, aplastic anemia
What is the tx for ITP?
PISD = initial tx: Prednisone 1-2 mg/kg/day; high dose IV Ig of 1 g/kg for 1-2 days for platelet counts < 5000; Splenectomy is definitive tx; Danazol 600 mg/day if all of above fail
A pt comes in with ptosis, diplopia, difficulty in chewing and swallowing, respiratory difficulties and limb weakness. The muscle weakness increases with activity and improves after periods of rest. What do they have?
Myasthenia Gravis (chronic autoimmune neuromuscular disorder with varying degrees of weakness of skeletal (voluntary) muscles of body)/ mya - muscles, anticholinesterase, acetylcholine receptor antibodies
In myasthenia gravis what do you see with the thymus gland?
it is abnormal (thymoma = thymic hyperplasia)/ it has clusters of immune cells indicative of lymphoid hyperplasia/ autoimmunity and production of the acetylcholine receptor antibodies setting the stage for attack on neuromuscular transmission -> skel muscle weakness
How do you dx myasthenia gravis?
lateral and ap x-rays of the chest and CT scans to demonstrate a co-existing thymoma; dx can be confirmed by response to a short-acting anticholinesterase (Phone Eddie)/ Edrophonium IV 10 mg will improve muscle strength, EMG, assay of serum for elevated levels of circulating acetylcholine receptor antibodies (ANA increased but lower titers than SLE)
How do you tx myasthenia gravis?
NAP: (since it improves with rest)/ otherwise CT
Anticholinesterase drugs give symptomatic relief
i.e. Neostigmine 15 mg qid and Pyridostigmine 60 mg qid; thymectomy; failure of above - corticosteroids
What is a group of vascular dz's that cause inflammatory injury and necrosis of the blood vessels? They are classified as small, medium or large.
What are the different types of systemic vasculitis?
PoG -> Wegman's for Japanese + German food = Polyarteritis Nodosa, Giant Cell Arteritis, Wegener's Granulomatosis, Takayasu's arteritis, Churg-Strauss Syndrome
What is a rare, systemic necrotizing inflammation of medium and small arteries that is mc in males? It is characterized by abdominal pain, systemic htn, jaundice, peripheral neuropathy and gangrene as well as livedo reticularis.
Polyarteritis Nodosa; poor prognosis
What should you screen for in pt's with polyarteritis nodosa?
Hep screening since 10% associated with Hep B
What has an ESR >50, is ANCA-negative, has low titers of ANA or RF?
How do you treat polyarteritis nodosa?
high dose corticosteroids (60 mg Prednisone daily), Methylprednisolone 1 g IV daily for 3 days if critically ill, immunosuppressive agents like Cyclophosphamide; Hep B responds to short course of prednisone + lamivudine 100 mg/d and plasmapheresis 3 wk for 6 weeks
What is a vasculitis that presents in pts over 50 with a warning headache, visual symptoms, jaw claudication, throat pain, dry cough, and a fever? It affects medium and large arteries. There is an asymmetry of pulses in arms, murmur of aortic regurgitation and bruits near the clavicle.
Giant Cell Arteritis (blindness if untreated)
What has an ESR > 50 or often 100 mm/h and an elevated AP?
Giant Cell Arteritis
How do you tx Giant Cell Arteritis?
urgently with prednisone 60 mg/d immediately -> bx to confirm/ then low dose ASA 81 mg/d/ continue prednisone 1 month before tapering
What is a rare disorder with vasculitis of small arteries and symptoms of nasal congestion, sinusitis and otitis media as well as fever, cough, dyspnea, wt loss and malaise? It is in males or females in their 4th-5th decades
Wegener's Granulomatosis (fatal if not treated)
What are PE findings for Wegener's Granulomatosis?
congestion, crusting, bleeding around nasal mucosa, inflamed gums/ otitis media, conjunctivitis, dvt and pe
What has lab findings of anemia, mild leukocytosis, elevated ESR, hilar adenopathy, C-ANCA (limited to kidney) and P-ANCA (localized to kidney)?
How do you tx Wegener's Granulomatosis?
crucial to prevent end-organ damage/ tightly control htn
CAP: Cyclophosphamide and prednisone, ASA 325 mg for TIAs/ AM: Azathioprine 2 mg/kg/d for remission and methotrexate 20 mg/wk if not sign/renal dysfunction
What is apulseless disease or pulse discrepance btwn extremities of > 30 mm Hg, aortic arch syndrome, young female arteritis of Asian descent? It affects large vessels and also are symptoms of fever, dizziness, fainting, stroke and night sweats.
How do you dx Takayasu's arteritis?
What is tx for Takayasu's arteritis?
P M & M: Prednisone 1 mg/kg/d tapered to < 20 in 8-20 wks with maintenance at 10 mg; Methotrexate 25 mg/wk and mycophenolate mofetil 1500 mg bid for those who are refractory
What has asthma as its cardinal feature and the pt also has nasal polyps and allergic rhinitis. This affects small to medium sized vessels and can also be associated with tingling in extremities, can affect the lungs, etc...
What are the Diagnostic Criteria for Churg-Strauss Syndrome?
PE MEAT: Paranasal sinus abnormalities, Eosinophilia > 10% on diff, Mononeuropathy, Extravascular eosinophils positive biopsy, Asthma, Transient pulmonary infiltrates
What is tx for Churg-Strauss Syndrome?
High dose prednisone 40-60 mg/d tapered slowly after 1 month; immunosuppressive agents