eMedicine Specialties > Dermatology > Connective Tissue Diseases
Mixed Connective Tissue Disease: Treatment & Medication
Updated: Oct 5, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
Medical care for mixed connective-tissue disease (MCTD) must be individualized and based on the severity and type of organ involvement. Plasmapheresis may be a successful treatment because of the removal of immune complexes in serum and improvement of visceral circulation.15
Consultations
Because of different presenting symptoms associated with mixed connective-tissue disease (MCTD), consultation with the following specialists should be considered:
- A dermatologist should evaluate the skin lesions.
- An internal medicine specialist should evaluate organ involvement.
Activity
Mixed connective-tissue disease (MCTD) patients have restricted mobility if joint and muscle pain is significant.
Medication
Mixed connective-tissue disease (MCTD) is a chronic and usually mild disease, which can be treated symptomatically or with corticosteroids or immunosuppressives if the severity of disease justifies it. Combined treatment allows dose reduction of systemic steroids (corticosteroid-sparing effect). Treatment depends on internal organ involvement. Usually middle doses of systemic corticosteroids are effective in most cases. Anti-inflammatory agents are helpful for arthralgia, myalgia, and swelling of the hands. Skin lesions can be treated with topical corticosteroids. In all cases, photoprotection is recommended.
Corticosteroids, systemic
These agents decrease autoimmune reactions, possibly by suppressing key components of the immune system. They are often used to treat collagen vascular diseases.
Prednisolone (Prelone Syrup, Pediapred Oral Solution, Delta-Cortef)
Synthetic adrenocortical steroid with predominantly glucocorticoid properties. Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reducing capillary permeability.
Adult
0.5-1 mg/kg/d PO, taper gradually as symptoms resolve
Pediatric
0.14-2 mg/kg/d (4-60 mg/m2/d) PO divided tid/qid
Coadministration with estrogens may decrease clearance; when used with digoxin, digitalis toxicity secondary to hypokalemia may increase; phenobarbital, phenytoin, and rifampin may increase the metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics; coadministration with ritonavir may significantly increase serum concentrations of prednisone; concomitant therapy with montelukast may result in severe peripheral edema; clarithromycin may increase risk of psychotic symptoms
Postmarketing surveillance reports indicate that risk of tendon rupture may be increased in patients receiving concomitant fluoroquinolones and corticosteroids, especially elderly patients; administration of asparaginase concurrently with or before prednisone therapy may result in increased toxicity
Documented hypersensitivity; viral, fungal, connective-tissue, or mycobacterial skin infections; peptic ulcer disease; hepatic dysfunction; GI disease
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
May unmask hypertension or diabetes or exacerbate peptic ulcer disease and tuberculosis; long-term sequelae associated with long-term steroid use include osteoporosis, cataracts, and pituitary-hypothalamic axis suppression; with high doses, patients may develop a steroid psychosis and are at increased risk of infections, particularly when oral steroids are used in conjunction with other immunosuppressants; frequently monitor patient's blood sugar level, blood pressure, and weight; monitor for Cushing syndrome
Immunosuppressants
These agents inhibit key factors that mediate immune reactions, which in turn decrease inflammatory responses.
Cyclosporine (Sandimmune, Gengraf, Neoral)
Cyclic polypeptide that suppresses some humoral immunity and, to a greater extent, cell-mediated immune reactions (eg, delayed hypersensitivity, allograft rejection, experimental allergic encephalomyelitis, and graft-vs-host disease) in a variety of organs. For children and adults, base dosing on ideal body weight. Onset of action is 1-2 mo; stabilization of disease may take 3-4 mo. Bioavailability not necessarily equal in different formulations; use caution. In severe disease, higher doses (transplant dosing) increases risk of adverse events and may be beyond the scope of practice for a general dermatologist.
Adult
Initial: 2.5 mg/kg/d PO divided bid; base dose on clinical response and tolerability; may increase dose by 0.5-0.75 mg/kg/d after 2 mo then monthly (not to exceed 4 mg/kg/d); decrease dose by 25-50% to control adverse events
Maintenance: Decrease to lowest possible dose to control disease
Pediatric
Administer as in adults
Carbamazepine, phenytoin, isoniazid, rifampin, and phenobarbital may decrease concentrations; azithromycin, itraconazole, nicardipine, ketoconazole, fluconazole, erythromycin, verapamil, grapefruit juice, diltiazem, aminoglycosides, acyclovir, amphotericin B, and clarithromycin may increase toxicity; acute renal failure, rhabdomyolysis, myositis, and myalgias increase when taken concurrently with lovastatin
Documented hypersensitivity; uncontrolled hypertension or malignancies; abnormal renal function; concomitant with PUVA or to a lesser extent UVB radiation, other immunosuppressive agents, coal tar, or radiation therapy may increase risk of skin cancer; patients potassium-sparing diuretics
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Baseline laboratory studies should include creatinine (on 2 occasions), BUN, CBC count, magnesium, potassium, uric acid, and lipid levels; evaluate CBC count, uric acid level, potassium level, lipid level, magnesium level, and renal and liver function often; evaluate BP frequently; if sustained HTN, sustained increase in serum creatinine level by 25% or more, or an abrupt increase in creatinine level by 50% or more, reduce dose; if change in creatinine level or HTN does not reverse after 2 (creatinine) or multiple (HTN) dose modifications, discontinue drug; caution in elderly patients or those with hyperkalemia; may increase risk of infection, lymphoma, and skin cancer; reserve IV use only for those who cannot take the PO form
Adverse effects include nephrotoxicity, hypertension, hepatotoxicity, gingival enlargement, hyperkalemia, hypomagnesemia, pancreatitis, and paresthesia; factors that may increase risk for neurotoxicity from cyclosporine include hypomagnesemia, hypocholesterolemia, fever, infection, hypertension, intravenous administration, and rapidly increasing cyclosporine blood levels; caution with medications metabolized by cytochrome P450 3A4
Nonsteroidal anti-inflammatory drugs
NSAIDs provide symptomatic relief for arthralgia, myalgia, edema, and tenderness.
Naproxen (Naprosyn, Anaprox)
For relief of mild to moderate pain; inhibits inflammatory reactions and pain by decreasing activity of cyclo-oxygenase, which decreases prostaglandin synthesis.
Adult
HCl formulation: 250-500 mg PO bid
Sodium formulation: 275-550 mg PO bid
May increase to 1.5 g/d of naproxen base for limited periods
Pediatric
<2 years: Not established
>2 years: 2.5-5 mg/kg/dose PO; not to exceed 15 mg/kg/d
Coadministration with aspirin increases risk of serious NSAID-related adverse effects; probenecid may increase concentrations and, possibly, toxicity of NSAIDs; may decrease effect of hydralazine, captopril, and beta-blockers; may decrease diuretic effects of furosemide and thiazides; may increase PT when taking anticoagulants (instruct patients to watch for signs of bleeding); may increase risk of methotrexate toxicity; may increase phenytoin levels when administered concurrently
Documented hypersensitivity including to aspirin or other NSAIDs; peptic ulcer disease; recent GI bleeding or perforation; renal insufficiency
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Category D in third trimester of pregnancy; acute renal insufficiency, interstitial nephritis, hyperkalemia, hyponatremia, and renal papillary necrosis may occur; patients with preexisting renal disease or compromised renal perfusion have risk of acute renal failure; leukopenia occurs rarely, is transient, and usually returns to normal during therapy; persistent leukopenia, granulocytopenia, or thrombocytopenia warrants further evaluation and may require discontinuation
Corticosteroids, topical
Topical corticosteroids are moderate or potent agents with anti-inflammatory and immunosuppressive properties. They can decrease epidermal proliferation.
Fluticasone (Cutivate)
Extremely potent vasoconstrictive and anti-inflammatory activities. Weak inhibitory affect on hypothalamic-pituitary-adrenocortical axis when applied topically. Other more potent topical corticosteroids may be useful for unresponsive inflammatory skin lesions. Less potent nonfluorinated topical corticosteroids may be useful in patients with less aggressive skin disease. Medium potency.
Adult
Apply sparingly to affected area bid; not to exceed 50 g/wk
Pediatric
<3 months: Not established
>3 months: Apply as in adults; use with caution
None reported
Documented hypersensitivity; viral, fungal, or bacterial skin infections
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Prolonged use, application over large surface areas, and use of potent steroids or occlusive dressings may increase systemic absorption and cause Cushing syndrome, reversible HPA-axis suppression, hyperglycemia, or glycosuria; fluorinated topical corticosteroids should not be applied to face, groin, or axilla or under occlusion in the typical patient because of possible cutaneous atrophy and telangiectasia
More on Mixed Connective Tissue Disease |
| Overview: Mixed Connective Tissue Disease |
| Differential Diagnoses & Workup: Mixed Connective Tissue Disease |
 Treatment & Medication: Mixed Connective Tissue Disease |
| Follow-up: Mixed Connective Tissue Disease |
| Multimedia: Mixed Connective Tissue Disease |
| References |
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References
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Further Reading
Keywords
mixed connective-tissue disease, MCTD, CTD, Sharp syndrome, Sharp's syndrome, systemic lupus erythematosus, SLE, systemic sclerosis, SSc, dermatomyositis, DM, polymyositis, PM, Sjögren syndrome, U1-ribonucleoprotein antibodies, U1-RNP
