Study guide
6.7 Systemic Sclerosis, Sjogren Syndrome & Inflammatory Myopathies
ABIM Internal Medicine Exam
Track your progress as you read
Finished sections get a check mark.
1 Chapter 0: The ABIM Exam, Blueprint & Study Strategy
0.1 Exam Format, Question Types & the 2026 Blueprint 0.2 Eligibility, Registration, Fees & Exam-Day Logistics 0.3 Scoring, the Passing Standard & Score Reports 0.4 Building a Blueprint-Weighted Study Plan
2 Chapter 1: Cardiovascular Disease (14% of Exam)
1.1 Ischemic Heart Disease & Acute Coronary Syndromes 1.2 Antithrombotic Therapy, Secondary Prevention & Mechanical Complications of MI 1.3 Atrial Fibrillation, Atrial Flutter & Stroke Prevention 1.4 Supraventricular Tachycardias, Ventricular Arrhythmias & Bradycardias 1.5 Heart Failure: Classification, Staging & Guideline-Directed Medical Therapy 1.6 Device Therapy, HFpEF & the Cardiomyopathies 1.7 Valvular Heart Disease & Endocarditis Prophylaxis 1.8 Pericardial Disease & Acute Aortic Syndromes 1.9 Hypertension: Classification, Diagnosis & Secondary Causes 1.10 Hypertensive Crises & Dyslipidemia 1.11 Adult Congenital Heart Disease & Coarctation of the Aorta 1.12 Syncope: Reflex, Orthostatic & Cardiac Causes 1.13 Cardiac Physical Examination, Murmurs & Antithrombotic Selection
3 Chapter 2: Endocrinology, Diabetes & Metabolism (9% of Exam)
2.1 Diabetes Mellitus: Diagnosis, Glycemic Targets & Pharmacotherapy 2.2 Insulin Therapy, Hyperglycemic Emergencies & Chronic Complications 2.3 Thyroid Function Testing & Hypothyroidism 2.4 Thyrotoxicosis, Thyroid Storm & Thyroid Nodules 2.5 Adrenal & Pituitary Disorders 2.6 Hypercalcemia, Hypocalcemia & Disorders of Mineral Metabolism 2.7 Osteoporosis & Paget Disease of Bone 2.8 Ovarian, Testicular & Reproductive Endocrine Disorders 2.9 Hypoglycemia, Endocrine Tumors, Polyglandular Syndromes & Severe Hypertriglyceridemia
4 Chapter 3: Gastroenterology & Hepatology (9% of Exam)
3.1 Esophageal Disease 3.2 Stomach & Duodenal Disease and Gastrointestinal Hemorrhage 3.3 Inflammatory Bowel Disease 3.4 Celiac Disease & Irritable Bowel Syndrome 3.5 Diverticular Disease & Colorectal Cancer Screening 3.6 Viral Hepatitis, Steatotic & Alcohol-Associated Liver Disease 3.7 Cirrhosis & the Complications of Portal Hypertension 3.8 Acute Pancreatitis 3.9 Chronic Pancreatitis & Biliary Tract Disease 3.10 Inherited, Autoimmune & Drug-Induced Liver Disease 3.11 Small Intestinal Disease, Ischemia & Gastrointestinal Complications of HIV
5 Chapter 4: Infectious Disease (9% of Exam)
4.1 Lower Respiratory Tract Infections & Sepsis Syndromes 4.2 Viral Respiratory Pathogens & Tuberculosis 4.3 Central Nervous System Infections 4.4 Skin, Soft Tissue, Bone & Joint Infections 4.5 Infective Endocarditis & Cardiovascular Infection 4.6 Clostridioides difficile & Catheter-Related Bloodstream Infection 4.7 HIV Diagnosis, Antiretroviral Therapy & Prevention 4.8 Opportunistic Infections & Sexually Transmitted Infections 4.9 Fever of Unknown Origin, Neutropenic Fever & Toxic Shock 4.10 Travel-Related Illness, Vector-Borne Disease & Outbreaks 4.11 Infection Prevention, Nosocomial Infection & Postexposure Prophylaxis 4.12 Rheumatic Fever, Poststreptococcal Disease, Enteric & Hepatic Infections
6 Chapter 5: Pulmonary Disease (9% of Exam)
5.1 COPD: Diagnosis, GOLD Classification & Initial Pharmacotherapy 5.2 Interventions That Reduce COPD Mortality & Exacerbation Management 5.3 Asthma: Diagnosis, Stepwise Therapy & Status Asthmaticus 5.4 Interstitial Lung Disease & Idiopathic Pulmonary Fibrosis 5.5 Sarcoidosis, Hypersensitivity Pneumonitis & Occupational Lung Disease 5.6 Pulmonary Embolism: Diagnosis, Risk Stratification & Anticoagulation 5.7 Pulmonary Hypertension & Sleep-Disordered Breathing 5.8 Acute Respiratory Distress Syndrome & Mechanical Ventilation 5.9 Pleural Disease, Empyema & Pneumothorax 5.10 Bronchiectasis, Cystic Fibrosis & Restrictive Lung Disease 5.11 Evaluation of Cough, Dyspnea, Hemoptysis & the Solitary Pulmonary Nodule
7 Chapter 6: Rheumatology & Orthopedics (9% of Exam)
6.1 Rheumatoid Arthritis: Diagnosis & Systemic Manifestations 6.2 Antirheumatic Pharmacotherapy & the Spondyloarthropathies 6.3 Gout: Pathophysiology & Acute Management 6.4 Urate-Lowering Therapy & Calcium Pyrophosphate Deposition Disease 6.5 Osteoarthritis 6.6 Systemic Lupus Erythematosus 6.7 Systemic Sclerosis, Sjogren Syndrome & Inflammatory Myopathies 6.8 The Vasculitides 6.9 Regional Musculoskeletal Syndromes, Back Pain & Sports Injuries 6.10 Other Primary Rheumatic Disorders & Mixed Connective Tissue Disease 6.11 Osteonecrosis & Metabolic Bone Disease
8 Chapter 7: Hematology (6% of Exam)
7.1 Approach to Anemia & the Microcytic Anemias 7.2 Macrocytic, Normocytic & Hemolytic Anemias 7.3 Hemoglobinopathies, Thalassemias & Paroxysmal Nocturnal Hemoglobinuria 7.4 Platelet Disorders, Thrombocytopenia & the Bleeding Patient 7.5 Coagulation Factor Disorders, Thrombophilia & Anticoagulant Reversal 7.6 Acute Leukemias, Chronic Leukemias & Myeloproliferative Neoplasms 7.7 Lymphomas & Plasma Cell Disorders 7.8 Myelodysplastic Syndrome, Myeloproliferative Neoplasms & Porphyria 7.9 Transfusion Medicine & Late Complications of Transplantation
9 Chapter 8: Medical Oncology (6% of Exam)
8.1 Breast, Lung, Colorectal & Prostate Cancer 8.2 Oncologic Emergencies 8.3 Genitourinary & Gynecologic Malignancies 8.4 Head and Neck, Bone & Soft Tissue Cancer and Cancer of Unknown Primary 8.5 Complications of Cancer & Its Treatment 8.6 Cancer Prevention, Survivorship & Psychosocial Care
10 Chapter 9: Nephrology & Urology (6% of Exam)
9.1 Acute Kidney Injury: Staging, Etiology & Urine Microscopy 9.2 Glomerular Disorders & Nephrotic Syndrome 9.3 Nephritic Syndromes, RPGN & Indications for Renal Replacement Therapy 9.4 Chronic Kidney Disease: Staging & Progression Retardation 9.5 Complications of Chronic Kidney Disease: Anemia, CKD-MBD & Acidosis 9.6 Hyponatremia, Hypernatremia & Water Balance 9.7 Potassium Disorders & Systematic Acid-Base Analysis 9.8 Renal Tubular Acidosis & Nephrolithiasis 9.9 Prostate Disorders, Urinary Incontinence & Other Urologic Conditions 9.10 Cystic Kidney Disease, Obstructive Uropathy, Tubulointerstitial Disease & Hematuria
11 Chapter 10: Neurology (4% of Exam)
10.1 Acute Ischemic Stroke: Syndromes & Reperfusion 10.2 Transient Ischemic Attack, Secondary Prevention & Hemorrhagic Stroke 10.3 Headache & Facial Pain 10.4 Seizures, Epilepsy & Movement Disorders 10.5 Multiple Sclerosis & Neuromuscular Junction Disorders 10.6 Motor Neuron Disease & Acute Peripheral Neuropathy 10.7 Cranial Neuropathies & Neuro-Ophthalmology 10.8 Peripheral Neuropathies, CNS Tumors & Other Neurologic Disorders
12 Chapter 11: Psychiatry & Behavioral Health (4% of Exam)
11.1 Major Depressive Disorder & Medical Mimics 11.2 Antidepressant Pharmacotherapy & Treatment-Resistant Depression 11.3 Bipolar Disorder, Mood Stabilizers & Lithium Toxicity 11.4 Anxiety, Stress-Related & Obsessive-Compulsive Disorders 11.5 Alcohol Use Disorder & Withdrawal 11.6 Pharmacotherapy for Alcohol & Opioid Use Disorder 11.7 Toxidromes, Psychiatric Emergencies & Suicide Risk Assessment 11.8 Psychotic, Somatic Symptom, Factitious & Personality Disorders 11.9 Eating Disorders, Sleep Disorders & Sexual Health
13 Chapter 12: Dermatology (3% of Exam)
12.1 Dermatitis, Papulosquamous & Inflammatory Dermatoses 12.2 Acne, Rosacea & Cutaneous Infections 12.3 Herpes Zoster & Autoimmune Blistering Diseases 12.4 Nodules, Tumors & Cutaneous Oncology 12.5 Dermatologic Emergencies & Severe Cutaneous Adverse Reactions 12.6 Pigment Disorders, Alopecia, Nail Disease & Photosensitivity 12.7 Vascular Dermatoses, Environmental Injury & Cutaneous Signs of Systemic Disease
14 Chapter 13: Obstetrics & Gynecology / Women's Health (3% of Exam)
13.1 Contraception, Preconception Care & Teratogenicity 13.2 Hypertensive Disorders of Pregnancy & Gestational Diabetes 13.3 Thyroid Disease & Venous Thromboembolism in Pregnancy 13.4 Menopause & Menopausal Hormone Therapy 13.5 Abnormal Uterine Bleeding, Endometrial Disease & Gynecologic Cancer Screening 13.6 Breast Disorders & Diseases of the Vulva and Vagina 13.7 Menstrual Disorders, Fibroids, Endometriosis & Early Pregnancy Complications
15 Chapter 14: Geriatric Syndromes (3% of Exam)
14.1 Comprehensive Geriatric Assessment & Frailty 14.2 Falls, Gait Assessment & Polypharmacy 14.3 Urinary Incontinence & Pressure Injuries in Older Adults 14.4 Mild Cognitive Impairment, Dementia & Its Subtypes 14.5 Delirium & the Pharmacotherapy of Cognitive Disorders 14.6 Palliative Care, Hospice & Advance Care Planning 14.7 Chronic Wounds, Nutrition, Swallowing, Constipation & Hip Fracture 14.8 Sensory Impairment, Dizziness, Elder Mistreatment & Care Settings
16 Chapter 15: Allergy & Immunology (2% of Exam)
15.1 Anaphylaxis: Recognition & Emergency Management 15.2 Urticaria & Angioedema 15.3 Drug Allergy, Desensitization & Adult Immunodeficiency 15.4 Food Allergy, Insect Venom Hypersensitivity, Serum Sickness & Contrast Reactions 15.5 Rhinitis, Conjunctivitis, Eosinophilic & Mast Cell Disorders
17 Chapter 16: Preventive Medicine, Immunization & Clinical Epidemiology
16.1 The USPSTF Framework & Evidence-Based Cancer Screening 16.2 Cardiovascular & Metabolic Screening and Primary Prevention 16.3 Adult Immunization & Vaccines in Special Populations 16.4 Clinical Epidemiology, Biostatistics & Study Design
18 Chapter 17: Miscellaneous - Ethics, Patient Safety & Quality Improvement (2% of Exam)
17.1 Principles of Bioethics & Decisional Capacity 17.2 Informed Consent, Treatment Refusal & Special Scenarios 17.3 Surrogate Decision-Making, Advance Directives & Confidentiality 17.4 Patient Safety Science, Medical Error & Diagnostic Reasoning 17.5 Quality Improvement Methods, Health Disparities & Health Equity
19 Chapter 18: Perioperative Medicine & Medical Consultation
18.1 Preoperative Cardiovascular Risk Assessment 18.2 Preoperative Pulmonary Risk & Perioperative Medication Management 18.3 Perioperative Anticoagulation, Bridging & Postoperative Complications
20 Chapter 19: Ophthalmology (1% of Exam)
19.1 The Acute Red Eye & Ophthalmic Emergencies 19.2 Painless Vision Loss & Chronic Ophthalmic Disease 19.3 Uveitis, Scleritis & Orbital Disease
21 Chapter 20: Otolaryngology & Dental Medicine (1% of Exam)
20.1 Dizziness, Vertigo & Epistaxis 20.2 Ear Disorders, Hearing Loss & Upper Respiratory Infections 20.3 Oral Cavity, Salivary Gland, Laryngeal & Taste and Smell Disorders
6.7 Systemic Sclerosis, Sjogren Syndrome & Inflammatory Myopathies
Key Takeaways Explain This Topic AI
Generalized scleroderma, CREST syndrome, scleroderma-associated interstitial lung disease and Raynaud phenomenon are the enumerated systemic sclerosis topics.
Anti-centromere antibody associates with limited cutaneous disease and pulmonary arterial hypertension, while anti-topoisomerase I associates with diffuse disease and interstitial lung disease.
Anti-RNA polymerase III antibody predicts scleroderma renal crisis and rapidly progressive skin thickening.
Angiotensin-converting enzyme inhibitors are the treatment of scleroderma renal crisis, and corticosteroids increase its risk.
Sjogren syndrome carries a markedly increased risk of non-Hodgkin lymphoma, so persistent parotid enlargement warrants evaluation.
Last updated: August 2026
- 1. Systemic Sclerosis (Scleroderma - SSc)
- Pathophysiology & Subsets
Systemic Sclerosis is a multisystem disorder characterized by a triad of: (1) microvascular endothelial cell injury and obliteration , (2) dysregulated immune activation , and (3) widespread progressive tissue fibrosis driven by hyperactive myofibroblasts overproducing collagen types I and III.
Feature Limited Cutaneous Systemic Sclerosis (lcSSc) Diffuse Cutaneous Systemic Sclerosis (dcSSc)
Skin Involvement Restricted to distal extremities (distal to elbows and knees) and face. Slow, indolent progression. Proximal extremities (arms, thighs) and trunk . Rapidly progressive skin thickening with early organ involvement.
CREST Syndrome C alcinosis cutis, R aynaud phenomenon (often precedes skin changes by years), E sophageal dysmotility, S clerodactyly, T elangiectasias. May have CREST features, plus widespread diffuse induration, tendon friction rubs, and joint contractures.
Characteristic Autoantibody Anti-Centromere Antibodies (ACA) positive (>60–80%). Anti-Scl-70 (Anti-Topoisomerase I) positive (30–40%) AND Anti-RNA Polymerase III positive (20%).
Primary Internal Organ Complications Isolated Pulmonary Arterial Hypertension (PAH - WHO Group 1) : Late complication occurring in 10-15%.; Severe esophageal aperistalsis and small bowel bacterial overgrowth. Interstitial Lung Disease (ILD / Pulmonary Fibrosis) : Correlates with Anti-Scl-70.; Scleroderma Renal Crisis (SRC) : Strongly correlates with Anti-RNA Polymerase III .
Surveillance Requirements Annual Transthoracic Echocardiogram, PFTs (disproportionately reduced DLCO with preserved FVC). Serial High-Resolution Chest CT (HRCT), PFTs (FVC and DLCO q6 months), daily home blood pressure monitoring.
Scleroderma Renal Crisis (SRC) — Emergency Management
Clinical Presentation: Rapid onset of malignant hypertension (SBP >180 mmHg, DBP >110 mmHg) , acute oliguric renal failure, microangiopathic hemolytic anemia ( MAHA with schistocytes and thrombocytopenia ), headache, encephalopathy, and retinopathy.
Major Precipitant: High-dose systemic corticosteroids (Prednisone >15 mg daily) . Board Rule: Avoid systemic glucocorticoids in diffuse systemic sclerosis whenever possible.
Definitive Treatment: Immediate initiation of short-acting ACE Inhibitors (Captopril) . Captopril is rapidly titrated upward to achieve blood pressure normalization. ACE inhibitors reverse hyperreninemic renal vasoconstriction and are life-saving.
Critical Board Rule: DO NOT discontinue or reduce ACE inhibitors if serum creatinine continues to rise during the initial days of SRC therapy! Continuation of ACE inhibitor therapy is mandatory for long-term renal survival and recovery.
Management of Raynaud Phenomenon & Digital Ulcers
Non-pharmacologic: Keep core body and extremities warm (layering, thermal gloves), avoidance of rapid temperature drops, strict smoking cessation , avoidance of sympathomimetics, decongestants, and non-selective beta-blockers.
First-line Pharmacotherapy: Dihydropyridine Calcium Channel Blockers (Amlodipine 5–10 mg daily or extended-release Nifedipine 30–60 mg daily) .
Second-line / Digital Ulcers: Phosphodiesterase-5 (PDE-5) Inhibitors (Sildenafil, Tadalafil) , topical nitroglycerin paste, or IV Prostacyclin analogues (Iloprost); Endothelin receptor antagonists (Bosentan) to prevent new digital ulcers.
2. Sjögren Syndrome
Pathophysiology: Chronic autoimmune lymphocytic infiltration (focal CD4+ T cells and B cells) of exocrine glands, resulting in glandular destruction, acinar atrophy, and secretory failure.
Clinical Features:
Keratoconjunctivitis Sicca (Dry Eyes): Foreign body sensation, burning, grittiness, photophobia. Diagnosed via Schirmer test (<5 mm wetting of filter paper at 5 minutes) or corneal rose bengal / lissamine green staining.
Xerostomia (Dry Mouth): Inability to swallow dry foods without liquids, severe rampant dental caries, burning oral mucosa, oral candidiasis.
Bilateral Parotid Gland Enlargement: Firm, nontender or tender swelling of salivary glands.
Extraglandular Features: Arthralgias, Raynaud phenomenon, autoimmune thyroiditis, distal renal tubular acidosis (Type 1 RTA with hypokalemia and nephrocalcinosis), and small-fiber sensory neuropathy.
Serologies & Pathology: Anti-Ro/SSA positive (~70%) and Anti-La/SSB positive (~50%); positive Rheumatoid Factor (70%) and hypergammaglobulinemia. Definitive diagnosis via labial minor salivary gland biopsy demonstrating focal lymphocytic sialadenitis ( focus score >= 1 focus of >=50 lymphocytes per 4 mm2 of glandular tissue ).
Malignancy Risk: 15- to 40-fold increased risk of B-cell Non-Hodgkin Lymphoma (most commonly MALT Lymphoma / Marginal Zone B-cell Lymphoma) . Warning signs requiring urgent lymphoma evaluation: persistent, rock-hard parotid enlargement, persistent lymphadenopathy, splenomegaly, palpable purpura, monoclonal paraprotein spike, or sudden loss of pre-existing RF positivity or new profound hypocomplementemia (low C4).
Management: Artificial tears, ocular cyclosporine drops, salivary substitutes, muscarinic agonists ( Pilocarpine, Cevimeline ) to stimulate salivary flow, meticulous dental hygiene, and Hydroxychloroquine for systemic arthralgias.
- 3. Inflammatory Myopathies
- Polymyositis (PM) vs. Dermatomyositis (DM) vs. Inclusion Body Myositis (IBM)
Feature Polymyositis (PM) Dermatomyositis (DM) Inclusion Body Myositis (IBM)
Demographics Adults (age 40–60), Female > Male. Adults (40–60) & Children (5–15), Female > Male. Men >50 years old (most common acquired myopathy in >50y).
Muscle Weakness Pattern Symmetric, proximal muscle weakness (shoulders, hip flexors; difficulty rising from chair, climbing stairs, lifting objects overhead, combing hair). Sensation is normal. Neck flexor weakness. Symmetric, proximal muscle weakness identical to PM, plus pathognomonic cutaneous lesions. Asymmetric, insidious weakness involving both distal and proximal muscles : predilection for deep finger flexors (forearm flexor digitorum profundus -> weak grip, dropping objects) and quadriceps (frequent falls, knee buckling). Preserved deltoids.
Cutaneous Findings None. 1. Gottron Papules : Violaceous flat papules over MCP, PIP, DIP joints.; 2. Heliotrope Rash : Violaceous erythema of upper eyelids with periorbital edema.; 3. Shawl Sign & V-Sign : Confluent macular erythema over upper back/shoulders (shawl) and anterior neck/upper chest (V-sign).; 4. Mechanic's Hands : Hyperkeratotic scaling/cracking along lateral fingers. None.
Laboratory Findings Marked Creatine Kinase (CK) elevation (10–50x normal) , elevated Aldolase, AST, ALT, LDH. Marked CK elevation (10–50x normal) , elevated Aldolase, AST, ALT, LDH. Normal or only mildly elevated CK (<3–5x normal) .
Autoantibodies Anti-Jo-1 (anti-histidyl-tRNA synthetase), Anti-SRP (severe necrotizing myopathy). Anti-Jo-1 (Anti-synthetase syndrome: myositis, ILD, Raynaud, mechanic's hands, arthritis).; Anti-Mi-2 (classic rash, good response).; Anti-TIF1-γ / Anti-NXP-2 (strongly associated with malignancy). Anti-cN1A (cytosolic 5'-nucleotidase 1A) in 30–50%.
Muscle Biopsy Pathology Endomysial infiltration by CD8+ cytotoxic T cells surrounding and invading non-necrotic muscle fibers with diffuse MHC Class I upregulation. Perivascular and perimysial infiltration by CD4+ T cells and B cells with perifascicular atrophy (microvascular complement C5b-9 membrane attack complex deposition). Endomysial inflammation with rimmed vacuoles containing amyloid and ubiquitin inclusions on modified Gomori trichrome stain.
Malignancy Association Modest increased risk. HIGH RISK of Occult Malignancy (3- to 6-fold increase) : Ovarian, breast, lung, colorectal, pancreatic cancer. Mandates comprehensive age- and sex-appropriate malignancy screening . No malignancy association.
Therapeutic Response First-line: High-dose Prednisone (1 mg/kg/day) + Methotrexate or Azathioprine. First-line: High-dose Prednisone (1 mg/kg/day) + Methotrexate or Azathioprine; IVIG for refractory disease/dysphagia. Refractory to corticosteroids and immunosuppressants ; management focuses on physical therapy, fall prevention, and swallowing evaluations.