A chapter specifically dedicated to the management of immunoinflammatory response developed in COVID-19 has been added.
Updates to all chapters of this clinical practice guideline for the diagnosis and treatment of systemic autoimmune diseases have been included.
The number of entities and complex clinical situations covered in the book has been increased.
The main therapeutic novelties that have arisen in the last four years have been introduced, especially on the rational use of new biological therapies.

Today, patients with systemic autoimmune diseases present complex diagnostic, therapeutic and prognostic problems in daily clinical practice which sometimes require difficult and risky decision-making. This clinical practical guideline supports the clinician in obtaining basic reference points, gaining quick experience and selecting the course of action to follow in each disease, based on the particular conditions of each patient.

This work will be a support guide for decision-making in this type of pathologies, essential for specialists in: Internal Medicine, Rheumatology, Pneumology, Cardiology, Dermatology, Endocrinology, Gastroenterology, Gynecology, Hematology, Immunology, Family and Community Medicine, Nephrology, Neurology, Ophthalmology and Otolaryngology.

The Spanish version of this book is available in this link.

SECTION I. Classic systemic autoimmune diseases

Systemic lupus erythematosu
Antiphospholipid syndrome
Sjögren disease
Systemic sclerosis
Idiopathic inflammatory myopathies

SECTION II. Systemic vasculitis

Systemic vasculitis: definition and classification
Giant cell arteritis. Polymyalgia rheumatica
Takayasu arteritis
Poliarteritis nodosa
Thromboangiitis obliterans (Buerger’s disease)
Kawasaki disease
Vasculitis associated with antineutrophil cytoplasmic antibodies (ANCA)
Granulomatosis with polyangiitis (Wegener’s), microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (Churg-Strauss)
IgA vasculitis (Henoch-Schönlein purpura)
Cryoglobulinemia
Urticarial vasculitis
Behçet’s disease.
Cogan syndrome
Single-organ (isolated or localized) vasculitis
Cutaneous leukocytoclastic vasculitis, cutaneous arteritis, primary central nervous system vasculitis, focal isolated vasculiti

SECTION III. Other systemic immunomediated diseases

Relapsing polychondritis
Sarcoidosis
Monogenic autoinflammatory diseases
Inborn errors of immunity (primary immunodeficiencies)
Hemophagocytic syndrome in adults
Adult Still’s disease
IgG4-related disease
Autoimmune/autoinflammatory syndrome induced by adjuvants (ASIA syndrome)
Immunoinflammatory response in COVID-19

SECTION IV. Complex situations related to systemic autoimmune diseases

Uveitis
Interstitial lung disease in autoimmune diseases
Extrahepatic manifestations of hepatitis C virus
Cardiovascular risk assessment in systemic autoimmune diseases
Fever in patients with systemic autoimmune diseases receiving immunosuppressive treatment
Autoimmune diseases and tropical infections
Perioperative control of the patient with systemic autoimmune disease
The patient with systemic autoimmune disease in the emergency department
Pregnancy and systemic autoimmune diseases
Autoimmune diseases triggered by biological treatments
Autoimmune manifestations related to antineoplastic biological therapies