Scleroderma - Revision history

ClaireLewis at 18:01, 16 October 2019

2019-10-16T18:01:48Z

← Older revision		Revision as of 18:01, 16 October 2019
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	***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		**Severe renal crisis is '''life threatening''' with incidence of 8-10% in Limited and 10-20% in diffuse
	***Similar to TTP/HUS with microangiography		***Similar to [[TTP]]/[[HUS]] with microangiography
	***Poor prognosis with sudden onset of [[hypertension]], [[encephalopathy]], [[CVA]], retinopathy		***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		***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		J. Emergency situations in rheumatology with a focus on systemic autoimmune
	diseases. Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub. 2016		diseases. Biomed Pap Med Fac Univ Palacky Olomouc Czech Repub. 2016
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	*Pulmonary		*Pulmonary
	**[[Respiratory failure]]		**[[Respiratory failure]]
	**Interstitial lung disease, leading to pulmonary fibrosis		**[[Interstitial lung disease]], leading to pulmonary fibrosis
	***Found in dorsal portion of both lower lobes, but can extend to upper lobes in severe diease		***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<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>		***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>
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	***Gradually progressive exercise breathlessness		***Gradually progressive exercise breathlessness
	***Fatigue		***Fatigue
	***Hoarseness from nerve palsy caused by dilated pulmonary artery stem		***[[dysphonia\|Hoarseness]] from nerve palsy caused by dilated pulmonary artery stem
	**[[Diffuse alveolar hemorrhage\|Alveolar hemorrhage]]		**[[Diffuse alveolar hemorrhage\|Alveolar hemorrhage]]
	***[[Hemoptysis]], infiltrates on CXR, anemia.		***[[Hemoptysis]], infiltrates on CXR, anemia.
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	***Can be from diastolic dysfunction, [[malignant hypertension]] during renal crisis, and decompensated [[pulmonary hypertension]]		***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		**Anginal [[chest pain\|pain]] from right ventricle ischemia and low cardiac output

	==Differential Diagnosis==		==Differential Diagnosis==
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	*Frequent blood pressure checks		*Frequent blood pressure checks
	*Serologic markers for each subset		*Serologic markers for each subset
	*[[UA]] for proteinuria or hematuria		*[[UA]] for [[proteinuria]] or [[hematuria]]
	*BMP		*BMP
	*[[CXR]], CT chest		*[[CXR]], CT chest
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	** [[ACEI]] drug of choice, e.g. [[captopril]] 6.25 to 12.5mg PO, TID.		** [[ACEI]] drug of choice, e.g. [[captopril]] 6.25 to 12.5mg PO, TID.
	*Avoid [[diuretics]]		*Avoid [[diuretics]]
	**Consult renal, as 50% of patients will require dialysis.		**Consult renal, as 50% of patients will require [[dialysis]].

	===[[Pulmonary hypertension]]===		===[[Pulmonary hypertension]]===
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	*Acute decompensation:		*Acute decompensation:
	**Optimize (usually reduce) RV preload:		**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>		***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		***[[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).		***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).
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	**Reduce RV afterload		**Reduce RV afterload
	***Avoid [[hypoxia]], maintain O2 sat >90% (increases pulmonary vasoconstriction)		***Avoid [[hypoxia]], maintain O2 sat >90% (increases pulmonary vasoconstriction)
	***Avoid ~~hypercapnea~~ (increases pulmonary vascular resistance)		***Avoid hypercapnia (increases pulmonary vascular resistance)
	***Avoid [[acidosis]]		***Avoid [[acidosis]]
	**Treat arrhythmias:'''		**Treat arrhythmias:'''
	***Avoid calcium channel blockers and β-blockers		***''Avoid'' calcium channel blockers and β-blockers
	**Optimize oxygenation		**Optimize oxygenation
	***Intubated patients should be optimized to increased O2 delivery and minimize ~~hypercapnea~~, maintain low tidal volumes and low PEEP as tolerated		***Intubated patients should be optimized to increased O2 delivery and minimize hypercapnia, maintain low tidal volumes and low PEEP as tolerated

	===Interstitial lung disease===		===Interstitial lung disease===
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	*Vessel embolization if localizable		*Vessel embolization if localizable
	*[[Hemoptysis\|Management of Hemoptysis]]		*[[Hemoptysis\|Management of Hemoptysis]]
			**[[Hemoptysis]], infiltrates on CXR, anemia.
			**Emergent bronchoscopy
			**High dose [[corticosteroids]], [[cyclophosphamide]], local vessel embolization or [[plasma exchange]]

	===CREST Syndrome===		===CREST Syndrome===
	*Empiric treatment with a [[PPI]] to prevent reflux and stricture formation		*Empiric treatment with a [[PPI]] to prevent reflux and stricture formation
	*Transfuse as needed for anemia due to mucosal telangiectasias		*Transfuse as needed for anemia due to mucosal telangiectasias
	*~~Ursodial~~ treatment if concomitant primary biliary cirrhosis to prevent progression to secondary cirrhosis		*[[Ursodiol]] 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==		==Disposition==

Mrosen5506: /* Clinical Features */

2018-08-12T20:25:38Z

Clinical Features

← Older revision		Revision as of 20:25, 12 August 2018
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	**hypo- or hyper- pigmentation of skin, extending from fingers and toes proximally, also involving chest and abdominal wall		**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 [[File:SclerodermaSaltPepperE3.jpg\|thumb\|Hypopigmentation in Diffuse Scleroderma, courtesy of [http://www.regionalderm.com Regional Derm website]]]		**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 phenomenon		**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		**Abrupt disease presentation; worse in first 18 months, then either improvement or worsening to involve internal organs
	*CREST Syndrome		*CREST Syndrome
	**Longstanding Raynaud's		**Longstanding [[Raynaud’s disease\|Raynaud's]]
	**Skin thickening and fibrosis distal to elbows and knees, and on face		**Skin thickening and fibrosis distal to elbows and knees, and on face
	**Subcutaneous calcinosis		**Subcutaneous calcinosis

Rossdonaldson1: /* CREST Syndrome */

2018-06-05T05:24:41Z

CREST Syndrome

← Older revision		Revision as of 05:24, 5 June 2018
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	===CREST Syndrome===		===CREST Syndrome===
	**Empiric treatment with a [[PPI]] to prevent reflux and stricture formation		*Empiric treatment with a [[PPI]] to prevent reflux and stricture formation
	**Transfuse as needed for anemia due to mucosal telangiectasias		*Transfuse as needed for anemia due to mucosal telangiectasias
	**Ursodial treatment if concomitant primary biliary cirrhosis to prevent progression to secondary cirrhosis		*Ursodial treatment if concomitant primary biliary cirrhosis to prevent progression to secondary cirrhosis


	***[[Hemoptysis]], infiltrates on CXR, anemia.		**[[Hemoptysis]], infiltrates on CXR, anemia.
	***Emergent bronchoscopy		**Emergent bronchoscopy
	***High dose [[corticosteroids]], [[cyclophosphamide]], local vessel embolization or [[plasma exchange]]		**High dose [[corticosteroids]], [[cyclophosphamide]], local vessel embolization or [[plasma exchange]]

	==Disposition==		==Disposition==

Rossdonaldson1: /* Differential Diagnosis */

2018-06-05T05:23:42Z

Differential Diagnosis

← Older revision		Revision as of 05:23, 5 June 2018
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	*[[Systemic Lupus Erythematosus]]		*[[Systemic Lupus Erythematosus]]
	*[[Sjögren syndrome]]		*[[Sjögren syndrome]]
	*Raynaud's Syndrome
			{{Blue digit DDX}}

	==Evaluation==		==Evaluation==

ClaireLewis: /* =CREST Syndrome */

2017-08-22T13:47:37Z

=CREST Syndrome

← Older revision		Revision as of 13:47, 22 August 2017
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	*[[Hemoptysis\|Management of Hemoptysis]]		*[[Hemoptysis\|Management of Hemoptysis]]

	===CREST Syndrome==		===CREST Syndrome===
	**Empiric treatment with a [[PPI]] to prevent reflux and stricture formation		**Empiric treatment with a [[PPI]] to prevent reflux and stricture formation
	**Transfuse as needed for anemia due to mucosal telangiectasias		**Transfuse as needed for anemia due to mucosal telangiectasias

ClaireLewis: /* =Interstitial lung disease */

2017-08-22T13:47:24Z

=Interstitial lung disease

← Older revision		Revision as of 13:47, 22 August 2017
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	***Intubated patients should be optimized to increased O2 delivery and minimize hypercapnea, maintain low tidal volumes and low PEEP as tolerated		***Intubated patients should be optimized to increased O2 delivery and minimize hypercapnea, maintain low tidal volumes and low PEEP as tolerated

	===Interstitial lung disease==		===Interstitial lung disease===
	*[[Steroids]]		*[[Steroids]]
	*[[Cyclophosphamide]]		*[[Cyclophosphamide]]

ClaireLewis at 13:46, 22 August 2017

2017-08-22T13:46:58Z

Show changes

ClaireLewis: /* Background */

2017-08-22T12:51:37Z

Background

← Older revision		Revision as of 12:51, 22 August 2017
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	==Background==		==Background==
	*Autoimmune [[collagen vascular]] ~~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

ClaireLewis: /* Background */

2017-08-22T12:51:23Z

Background

← Older revision		Revision as of 12:51, 22 August 2017
Line 1:		Line 1:
	==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

Taleesha: /* Background */

2017-08-21T17:03:36Z

Background

← Older revision		Revision as of 17:03, 21 August 2017
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	**Vascular wall thickening		**Vascular wall thickening
	**Narrowed lumen		**Narrowed lumen
			*Two types, Diffuse and Limited
			**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)
	*~~Skin lesions (~~fingers, ~~hands~~, ~~face)~~		*Diffuse Systemic Sclerosis
	*~~Carpal tunnel~~		**hypo- or hyper- pigmentation of skin, extending from fingers and toes proximally, also involving chest and abdominal wall
	*Raynaud's ~~phenomenon~~		**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]]]
	*~~Interstitial lung disease~~		**Symmetric hand edema and Raynaud's phenomenon
	*~~Renal impairment~~		**Abrupt Disease presentation; worse in first 18 months then improvement or worsening to involve internal organs
	*GI dysmotility		*Liminted Sytemic Sclerosis, aka "CREST Syndrome"
	*~~[[GERD]]/aspiration~~		**Longstanding Raynaud's
	*~~Chronic [[esophagitis]] and stricture formation~~		**Skin thickening and fibrosis distal to elbows and knees, and on face
			**Subcutaneous calcinosis
			**Esophageal dysmotility
			**Sclerodactyly
			**Telangiectasia
			**Indolent course

	===Emergencies===		===Emergencies===
	*~~Scleroderma renal crisis~~		*Renal Crisis
	**Causes [[hypertensive emergency]]		**Causes are vessel narrowing and subsequent ischemic kidney disease
			**Lead to [[hypertensive emergency]], [[acute renal failure]], and microangiopathic hemolytic anemia
			**Occurs during first 5 years of the disease
	**Treatment is strict BP control - [[ACEI]] drug of choice		**Treatment is strict BP control - [[ACEI]] drug of choice
	***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, cyclosporin 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
			**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<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>
	**[[ARDS]]		**[[ARDS]]
	**[[Aspiration pneumonitis]]		**[[Aspiration pneumonitis]]
	**[[Pulmonary hypertension]]		**[[Pulmonary hypertension]]
			***Gradually progressive exercise breathlessness
			***Fatigue
			***Anginal pain from right ventricle ischemia and low cardiac output
			***Hoarseness from nerve palsy caused by dilated pulmonary artery stem
	**[[Diffuse alveolar hemorrhage\|Alveolar hemorrhage]]		**[[Diffuse alveolar hemorrhage\|Alveolar hemorrhage]]
	***[[Hemoptysis]], infiltrates on CXR, anemia.		***[[Hemoptysis]], infiltrates on CXR, anemia.
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	==Differential Diagnosis==		==Differential Diagnosis==
			*[[Systemic Lupus Erythematous]]
			*Sjogren's Syndrome
			*Raynaud's Syndrome

	==Evaluation==		==Evaluation==
			*Frequent blood pressure checks
			*Serologic markers for each subset
			*Renal Crisis
			**Urinalysis for proteinuria or hematuria
			**Basic Metabolic Panel
			**Chest Xray for pulmonary edema
			*Pulmonary Arterial Hypertension
			**Basic labs, including troponin
			**Echo to evaluate right heart and estimation of right atrial pressure
			**Right heart catheterization indicated in symptomatic patients
			*Interstitial Lung Diease
			**Frequent checks of FVC to monitor disease
			**Chest xray, CT of the chest
			**Lung biopsy
			*CREST Syndrome
			**Endoscopy and Hemoccult test to evaluate for blood loss from mucosal telangiectasias (gastric antral vascular ectasia, described as "watermelon stomach."


	==Management==		==Management==
			*Renal Crisis
			**Rapid control of blood pressure
			**Start on ACE Inhibitor, captopril 6.25 to 12.5mg PO, TID.
			**Avoid diuretics
			**Consult Nephrology, as 50% of patients will require dialysis.
			*Pulmonary Arterial Hypertension
			**Warfarin to prevent [[Pulmonary Embolism]]
			**Endothelin-I receptor antagonist, phosphodiesterase inhibitor or IV prostanoids
			*Interstitial Lung Disease
			**lower dose glucocorticoid
			**cyclophosphamide
			**May require ventilatory support (NIPPV or Intubation)
			*CREST Syndrome
			**Empiric treatment with a PPI to prevent reflux and stricture formation
			**Blood replacement if chronic loss from mucosal telangiectasias
			**Ursodial treatment if concomitant primary biliary cirrhosis to prevent progression to secondary cirrhosis

	==Disposition==		==Disposition==
			*Hospitalization for any elevation in blood pressure, difficulty breathing, evidence of heart strain or pulmonary edema.
			*Consult Rheumatology
			*Consult Pulmonary
			*Consult Nephrology

	==See Also==		==See Also==