Scleroderma: Difference between revisions
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== Background == | ==Background== | ||
*Autoimmune disease, aka '''Systemic Sclerosis''' | *Autoimmune [[collagen vascular disease]], aka '''Systemic Sclerosis''' | ||
*Inappropriate and excessive accumulation of collagen and matrix in various tissues | *Inappropriate and excessive accumulation of collagen and matrix in various tissues | ||
*Widespread vascular lesions | *Widespread vascular lesions | ||
Line 7: | Line 7: | ||
**Vascular wall thickening | **Vascular wall thickening | ||
**Narrowed lumen | **Narrowed lumen | ||
*Two types: | |||
**Diffuse Systemic Sclerosis: skin changes, which can progress to internal organ involvement | |||
**Limited Cutaneous Systemic Sclerosis, aka "CREST Syndrome" | |||
== Clinical Features == | ==Clinical Features== | ||
*Systemic complaints (fever, malaise, fatigue, weight loss, myalgias) | *Systemic complaints ([[fever]], malaise, [[fatigue]], weight loss, myalgias) | ||
* | *Diffuse Systemic Sclerosis | ||
* | **hypo- or hyper- pigmentation of skin, extending from fingers and toes proximally, also involving chest and abdominal wall | ||
*Raynaud's | **Pigment is perserved around hair follicles leading to ''salt and pepper'' appearance [[File:SclerodermaSaltPepperE3.jpg|thumb|Hypopigmentation in Diffuse Scleroderma, courtesy of [http://www.regionalderm.com Regional Derm website]]] | ||
* | **Symmetric hand edema and [[Raynaud’s disease|Raynaud's]] phenomenon | ||
* | **Abrupt disease presentation; worse in first 18 months, then either improvement or worsening to involve internal organs | ||
* | *CREST Syndrome | ||
* | **Longstanding [[Raynaud’s disease|Raynaud's]] | ||
* | **Skin thickening and fibrosis distal to elbows and knees, and on face | ||
**Subcutaneous calcinosis | |||
**Esophageal dysmotility | |||
**Sclerodactyly | |||
**Telangiectasia | |||
**Indolent course | |||
== Emergencies == | ===Emergencies=== | ||
* | *Renal crisis | ||
**Causes | **Causes are vessel narrowing and subsequent ischemic kidney disease | ||
** | **Leads to [[hypertensive emergency]], [[acute renal failure]], and [[microangiopathic hemolytic anemia]] | ||
**Occurs during first 5 years of the disease | |||
***Was the most common cause of death prior to ACEi usage<ref>Komocsi A, Vorobcsuk A, Faludi R, et al. The impact of cardiopulmonary manifestations on the mortality of SSc: a systematic review and meta-analysis of observational studies. Rheumatology (Oxford) 2012;51:1027–1036</ref> | ***Was the most common cause of death prior to ACEi usage<ref>Komocsi A, Vorobcsuk A, Faludi R, et al. The impact of cardiopulmonary manifestations on the mortality of SSc: a systematic review and meta-analysis of observational studies. Rheumatology (Oxford) 2012;51:1027–1036</ref> | ||
**Severe renal crisis is '''life threatening''' with incidence of 8-10% in Limited and 10-20% in diffuse | |||
***Similar to TTP/HUS with microangiography | |||
***Poor prognosis with sudden onset of [[hypertension]], [[encephalopathy]], [[CVA]], retinopathy | |||
***Risk factors are rapidly progressing diffuse scleroderma, high dose glucocorticoid use, cyclosporine therapy, presence of anti-RNA-polymerase antibodies.<ref>Vymetal J, Skacelova M, Smrzova A, Klicova A, Schubertova M, Horak P, Zadrazil | |||
J. Emergency situations in rheumatology with a focus on systemic autoimmune | |||
diseases. Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub. 2016 | |||
Mar;160(1):20-9. doi: 10.5507/bp.2016.002. Epub 2016 Feb 10. Review. PubMed PMID: | |||
26868300.</ref> | |||
*Pulmonary | *Pulmonary | ||
**Respiratory failure | **[[Respiratory failure]] | ||
**ARDS | **Interstitial lung disease, leading to pulmonary fibrosis | ||
**Aspiration pneumonitis | ***Found in dorsal portion of both lower lobes, but can extend to upper lobes in severe diease | ||
**Pulmonary | ***Poor prognosis with severe pulmonary impairment. 42% die within 10 years of disease onset<ref>Luo Y, Xiao R. Interstitial Lung Disease in Scleroderma: Clinical Features and Pathogenesis. Rheumatology 2011 doi:10.4172/2161- 1149. S1-002. Available from: http://omicsonline.org/ interstitial-lung-disease-in-scleroderma-clinical-features-and- pathogenesis-2161-1149.S1-002.pdf</ref> | ||
**Alveolar hemorrhage | **[[ARDS]] | ||
**[[Aspiration pneumonitis]] | |||
**[[Pulmonary hypertension]] | |||
***Gradually progressive exercise breathlessness | |||
***Fatigue | |||
***Hoarseness from nerve palsy caused by dilated pulmonary artery stem | |||
**[[Diffuse alveolar hemorrhage|Alveolar hemorrhage]] | |||
***[[Hemoptysis]], infiltrates on CXR, anemia. | ***[[Hemoptysis]], infiltrates on CXR, anemia. | ||
*Cardiac | *Cardiac | ||
**Acute heart failure | **Acute [[heart failure]] | ||
***Can be from diastolic dysfunction, malignant | ***Can be from diastolic dysfunction, [[malignant hypertension]] during renal crisis, and decompensated [[pulmonary hypertension]] | ||
**'''Cardiopulmonary''' complications are the most common causes of scleroderma-related death<ref>Nikpour, M., Baron, M. Mortality in systemic sclerosis: lessons learned from population-based and observational cohort studies. Curr Opin Rheumatol. 2014;26:131–137</ref>, with cardiac causes<ref>Komocsi A, Vorobcsuk A, Faludi R, et al. The impact of cardiopulmonary manifestations on the mortality of SSc: a systematic review and meta-analysis of observational studies. Rheumatology (Oxford) 2012;51:1027–1036</ref> and pulmonary fibrosis<ref>Tyndall AJ, Bannert B, Vonk M, Airo P, Cozzi F, Carreira PE, et al. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis. 2010;69:1809–15. doi: 10.1136/ard.2009.114264</ref> being the most common. | **'''Cardiopulmonary''' complications are the most common causes of scleroderma-related death<ref>Nikpour, M., Baron, M. Mortality in systemic sclerosis: lessons learned from population-based and observational cohort studies. Curr Opin Rheumatol. 2014;26:131–137</ref>, with cardiac causes<ref>Komocsi A, Vorobcsuk A, Faludi R, et al. The impact of cardiopulmonary manifestations on the mortality of SSc: a systematic review and meta-analysis of observational studies. Rheumatology (Oxford) 2012;51:1027–1036</ref> and pulmonary fibrosis<ref>Tyndall AJ, Bannert B, Vonk M, Airo P, Cozzi F, Carreira PE, et al. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis. 2010;69:1809–15. doi: 10.1136/ard.2009.114264</ref> being the most common. | ||
**Anginal pain from right ventricle ischemia and low cardiac output | |||
==Differential Diagnosis== | |||
*[[Systemic Lupus Erythematosus]] | |||
*[[Sjögren syndrome]] | |||
{{Blue digit DDX}} | |||
==Evaluation== | |||
*Frequent blood pressure checks | |||
*Serologic markers for each subset | |||
*[[UA]] for proteinuria or hematuria | |||
*BMP | |||
*[[CXR]], CT chest | |||
* +/- echo, right heart cath, lung biopsy | |||
*CREST syndrome | |||
**May need endoscopy and Hemoccult test to evaluate for blood loss from mucosal telangiectasias (gastric antral vascular ectasia, described as "watermelon stomach." | |||
==Management== | |||
===Renal crisis=== | |||
*Rapid control of blood pressure | |||
** [[ACEI]] drug of choice, e.g. [[captopril]] 6.25 to 12.5mg PO, TID. | |||
*Avoid [[diuretics]] | |||
**Consult renal, as 50% of patients will require dialysis. | |||
===[[Pulmonary hypertension]]=== | |||
*Chronic management may include prostaglandin derivatives (e.g. [[epoprostenol]]), PDE-5 inhibitors (e.g. [[sildenafil]]), and/or endothelin receptor antagonists (e.g. [[bosentan]]) | |||
*Acute decompensation: | |||
**Optimize (usually reduce) RV preload: | |||
***Usually euvolemic or hypervolemic, rarely need IV fluids so diuretics can benefit and treat the RV failure<ref>Ternacle, J et al. Diruetics in Normotensive Patients with Acute Pulmonary Embolism and Right Ventricular Dilation. Circulation Journal. Vol 77(10) 2013. 2612-618.</ref> | |||
***[[Furosemide]] 20-40mg IV or drip at 5-20 mg/hr | |||
***If suspect [[sepsis]] or [[hypovolemia]], small (250-500cc) NS challenge to assess fluid responsiveness. If not responsive to IVF challenge, start [[norepinephrine]] (MAP > 65 mmHg). | |||
**Increase cardiac output | |||
***Once MAP >65 mmHg, start low dose [[dobutamine]] (5-10mcg/kg/min) | |||
***Improves inotropic support and theoretically decreases pulmonary vascular resistance | |||
**Reduce RV afterload | |||
***Avoid [[hypoxia]], maintain O2 sat >90% (increases pulmonary vasoconstriction) | |||
***Avoid hypercapnea (increases pulmonary vascular resistance) | |||
***Avoid [[acidosis]] | |||
**Treat arrhythmias:''' | |||
***Avoid calcium channel blockers and β-blockers | |||
**Optimize oxygenation | |||
***Intubated patients should be optimized to increased O2 delivery and minimize hypercapnea, maintain low tidal volumes and low PEEP as tolerated | |||
===Interstitial lung disease=== | |||
*[[Steroids]] | |||
*[[Cyclophosphamide]] | |||
*Ventilatory support prn | |||
===[[Diffuse alveolar hemorrhage|Alveolar hemorrhage]]=== | |||
*Airway stabilization if large volume [[hemoptysis]] or problems with oxygenation/ventilation (>8.0 ETT preferable) | |||
*IV corticosteroids - high dose [[Corticosteroids|Methylprednisolone]] Q6H<ref>Park M. Diffuse Alveolar Hemorrhage. Tuberc Resp Dis (Seoul) 2013. 74(4):151-162</ref> or [[cyclophosphamide]] | |||
*Bronchoscopy for vessel localization | |||
*Vessel embolization if localizable | |||
*[[Hemoptysis|Management of Hemoptysis]] | |||
===CREST Syndrome=== | |||
*Empiric treatment with a [[PPI]] to prevent reflux and stricture formation | |||
*Transfuse as needed for anemia due to mucosal telangiectasias | |||
*Ursodial treatment if concomitant primary biliary cirrhosis to prevent progression to secondary cirrhosis | |||
**[[Hemoptysis]], infiltrates on CXR, anemia. | |||
**Emergent bronchoscopy | |||
**High dose [[corticosteroids]], [[cyclophosphamide]], local vessel embolization or [[plasma exchange]] | |||
==Disposition== | |||
*Hospitalization for any elevation in blood pressure, difficulty breathing, evidence of heart strain or pulmonary edema. | |||
==See Also== | |||
*[[Collagen vascular disease]] | |||
==References== | ==References== | ||
<references/> | <references/> | ||
[[Category: | [[Category:Rheumatology]] |
Revision as of 20:25, 12 August 2018
Background
- Autoimmune collagen vascular disease, aka Systemic Sclerosis
- Inappropriate and excessive accumulation of collagen and matrix in various tissues
- Widespread vascular lesions
- Endothelial dysfunction
- Vascular spasm
- Vascular wall thickening
- Narrowed lumen
- Two types:
- Diffuse Systemic Sclerosis: skin changes, which can progress to internal organ involvement
- Limited Cutaneous Systemic Sclerosis, aka "CREST Syndrome"
Clinical Features
- Systemic complaints (fever, malaise, fatigue, weight loss, myalgias)
- Diffuse Systemic Sclerosis
- hypo- or hyper- pigmentation of skin, extending from fingers and toes proximally, also involving chest and abdominal wall
- Pigment is perserved around hair follicles leading to salt and pepper appearance
- Symmetric hand edema and Raynaud's phenomenon
- Abrupt disease presentation; worse in first 18 months, then either improvement or worsening to involve internal organs
- CREST Syndrome
- Longstanding Raynaud's
- Skin thickening and fibrosis distal to elbows and knees, and on face
- Subcutaneous calcinosis
- Esophageal dysmotility
- Sclerodactyly
- Telangiectasia
- Indolent course
Emergencies
- Renal crisis
- Causes are vessel narrowing and subsequent ischemic kidney disease
- Leads to hypertensive emergency, acute renal failure, and microangiopathic hemolytic anemia
- Occurs during first 5 years of the disease
- Was the most common cause of death prior to ACEi usage[1]
- Severe renal crisis is life threatening with incidence of 8-10% in Limited and 10-20% in diffuse
- Similar to TTP/HUS with microangiography
- Poor prognosis with sudden onset of hypertension, encephalopathy, CVA, retinopathy
- Risk factors are rapidly progressing diffuse scleroderma, high dose glucocorticoid use, cyclosporine therapy, presence of anti-RNA-polymerase antibodies.[2]
- Pulmonary
- Respiratory failure
- Interstitial lung disease, leading to pulmonary fibrosis
- Found in dorsal portion of both lower lobes, but can extend to upper lobes in severe diease
- Poor prognosis with severe pulmonary impairment. 42% die within 10 years of disease onset[3]
- ARDS
- Aspiration pneumonitis
- Pulmonary hypertension
- Gradually progressive exercise breathlessness
- Fatigue
- Hoarseness from nerve palsy caused by dilated pulmonary artery stem
- Alveolar hemorrhage
- Hemoptysis, infiltrates on CXR, anemia.
- Cardiac
- Acute heart failure
- Can be from diastolic dysfunction, malignant hypertension during renal crisis, and decompensated pulmonary hypertension
- Cardiopulmonary complications are the most common causes of scleroderma-related death[4], with cardiac causes[5] and pulmonary fibrosis[6] being the most common.
- Anginal pain from right ventricle ischemia and low cardiac output
- Acute heart failure
Differential Diagnosis
Blue Digit
- Acute arterial ischemia
- Atheroembolism (AKA Blue Toe Syndrome)
- Arterial embolism
- Arterial thrombosis
- Vasospastic Disorders
- Raynaud’s disease
- Primary erythromelalgia
- Autoimmune
- Idiopathic
- Thromboangiitis obliterans (Buerger's disease)
- Chronic peripheral artery disease
- Atherosclerosis obliterans
Evaluation
- Frequent blood pressure checks
- Serologic markers for each subset
- UA for proteinuria or hematuria
- BMP
- CXR, CT chest
- +/- echo, right heart cath, lung biopsy
- CREST syndrome
- May need endoscopy and Hemoccult test to evaluate for blood loss from mucosal telangiectasias (gastric antral vascular ectasia, described as "watermelon stomach."
Management
Renal crisis
- Rapid control of blood pressure
- Avoid diuretics
- Consult renal, as 50% of patients will require dialysis.
Pulmonary hypertension
- Chronic management may include prostaglandin derivatives (e.g. epoprostenol), PDE-5 inhibitors (e.g. sildenafil), and/or endothelin receptor antagonists (e.g. bosentan)
- Acute decompensation:
- Optimize (usually reduce) RV preload:
- Usually euvolemic or hypervolemic, rarely need IV fluids so diuretics can benefit and treat the RV failure[7]
- Furosemide 20-40mg IV or drip at 5-20 mg/hr
- If suspect sepsis or hypovolemia, small (250-500cc) NS challenge to assess fluid responsiveness. If not responsive to IVF challenge, start norepinephrine (MAP > 65 mmHg).
- Increase cardiac output
- Once MAP >65 mmHg, start low dose dobutamine (5-10mcg/kg/min)
- Improves inotropic support and theoretically decreases pulmonary vascular resistance
- Reduce RV afterload
- Treat arrhythmias:
- Avoid calcium channel blockers and β-blockers
- Optimize oxygenation
- Intubated patients should be optimized to increased O2 delivery and minimize hypercapnea, maintain low tidal volumes and low PEEP as tolerated
- Optimize (usually reduce) RV preload:
Interstitial lung disease
- Steroids
- Cyclophosphamide
- Ventilatory support prn
Alveolar hemorrhage
- Airway stabilization if large volume hemoptysis or problems with oxygenation/ventilation (>8.0 ETT preferable)
- IV corticosteroids - high dose Methylprednisolone Q6H[8] or cyclophosphamide
- Bronchoscopy for vessel localization
- Vessel embolization if localizable
- Management of Hemoptysis
CREST Syndrome
- Empiric treatment with a PPI to prevent reflux and stricture formation
- Transfuse as needed for anemia due to mucosal telangiectasias
- Ursodial treatment if concomitant primary biliary cirrhosis to prevent progression to secondary cirrhosis
- Hemoptysis, infiltrates on CXR, anemia.
- Emergent bronchoscopy
- High dose corticosteroids, cyclophosphamide, local vessel embolization or plasma exchange
Disposition
- Hospitalization for any elevation in blood pressure, difficulty breathing, evidence of heart strain or pulmonary edema.
See Also
References
- ↑ Komocsi A, Vorobcsuk A, Faludi R, et al. The impact of cardiopulmonary manifestations on the mortality of SSc: a systematic review and meta-analysis of observational studies. Rheumatology (Oxford) 2012;51:1027–1036
- ↑ Vymetal J, Skacelova M, Smrzova A, Klicova A, Schubertova M, Horak P, Zadrazil J. Emergency situations in rheumatology with a focus on systemic autoimmune diseases. Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub. 2016 Mar;160(1):20-9. doi: 10.5507/bp.2016.002. Epub 2016 Feb 10. Review. PubMed PMID: 26868300.
- ↑ Luo Y, Xiao R. Interstitial Lung Disease in Scleroderma: Clinical Features and Pathogenesis. Rheumatology 2011 doi:10.4172/2161- 1149. S1-002. Available from: http://omicsonline.org/ interstitial-lung-disease-in-scleroderma-clinical-features-and- pathogenesis-2161-1149.S1-002.pdf
- ↑ Nikpour, M., Baron, M. Mortality in systemic sclerosis: lessons learned from population-based and observational cohort studies. Curr Opin Rheumatol. 2014;26:131–137
- ↑ Komocsi A, Vorobcsuk A, Faludi R, et al. The impact of cardiopulmonary manifestations on the mortality of SSc: a systematic review and meta-analysis of observational studies. Rheumatology (Oxford) 2012;51:1027–1036
- ↑ Tyndall AJ, Bannert B, Vonk M, Airo P, Cozzi F, Carreira PE, et al. Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database. Ann Rheum Dis. 2010;69:1809–15. doi: 10.1136/ard.2009.114264
- ↑ Ternacle, J et al. Diruetics in Normotensive Patients with Acute Pulmonary Embolism and Right Ventricular Dilation. Circulation Journal. Vol 77(10) 2013. 2612-618.
- ↑ Park M. Diffuse Alveolar Hemorrhage. Tuberc Resp Dis (Seoul) 2013. 74(4):151-162